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Localized Scleroderma Is Indeed LOCALIZED Skin, but May Involve Various Joints, Such As the Such As Thickening of the Skin on All the Fingers Possible

Localized Scleroderma Is Indeed LOCALIZED Skin, but May Involve Various Joints, Such As the Such As Thickening of the Skin on All the Fingers Possible

() during the course of their . The It’s important to look for physical signs of the or functional deformity. However, stabilization Our Three-Fold Mission Is is not limited to beneath the involved internal type of (systemic sclerosis), (inactive disease) of localized scleroderma is indeed LOCALIZED , but may involve various joints, such as the such as thickening of the skin on all the fingers possible. The outlook and of localized Support, Education and knees, wrists and even the spine. (), and thickening of the skin on the scleroderma is generally quite favorable. First, the Research SCLERODERMA Occasionally, this complication occurs before the (without color changes). There are other clues disease is not fatal, does not appear to decrease appearance of in the skin, causing some that can be identified during the initial examination. lifespan and, except for extremely rare situations, is confusion with rheumatoid for a short For example, patients with localized scleroderma not associated with another disease that has internal period of time. Eventually, the subside, do not have the typical changes at the base of the involvement. Secondly, in most instances even in the face of new skin involvement but an fingernails that are common in the internal (systemic the condition is self-limited, and this definitely association with spine arthritis has been reported. sclerosis) disease: tiny changes that applies to individual areas of . In those Along the same lines, it is highly unlikely that require a special magnifying lens for proper cases, no treatment is required or advisable, except patients with localized scleroderma progress and detection. Another important question is whether for some emollients or, in some cases, mild topical develop severe systemic (internal) conditions such patients have what is called Raynaud’s phenomenon. steroids. Therefore, systemic treatment with a as , or erythematosus, This refers to the inability of the patient’s hands to or other treatment interventions (for or systemic sclerosis (the internal form of withstand cold exposure, so that the fingers may be example, light), are reserved for more scleroderma). Still, it is important to recognize that come pale, or even experience numbness and pain, extensive forms of localized scleroderma. There morphea and other types of localized scleroderma and/or develop a temporary bluish appearance with are many publications that reference favorable may be present in several of these systemic even mild cold temperatures, or when touching results when using certain , but these conditions, including systemic , a cold beverage, or getting some item from the have truly not been proven to work. Examples of systemic sclerosis, and blistering . An freezer. Patients with localized scleroderma these medications include: (Dilantin), association has been found with a parasitic disease generally do not have Raynaud’s phenomenon, systemic (cortisone), antimalarial (toxoplasma). While this does not imply that patients which is present in the majority of patients with drugs (plaquenil, chloroquine), cyclosporine, with localized scleroderma evolve to develop these systemic sclerosis. d-, antibiotics (presumably to treat an conditions, it does emphasize the need for follow-up underlying problem.) and E. and periodic evaluations, especially in patients who Several blood tests may be performed, which, when A previously used -based (with continue to have active skin problems or develop abnormal, help to determine how active the disease calcitriol) has now been proven not to work. There new issues. is and how extensive or prolonged it may become. is some recent good news for patients with extensive These blood tests include the number of blood disease who require proven and other Are There Any Tests to (cell normally present in our blood), the interventions. (as an anti-inflammatory Prove the Diagnosis of level of blood immune (immunoglobulins), drug) has proved effective in children with active and various blood auto-: antinuclear and extensive (and deep) morphea. It has some side Localized Scleroderma? antibodies (ANA), antibodies to single-stranded effects and should only be used when truly necessary. The diagnosis of localized scleroderma is mainly DNA (ssDNA) and antihistones antibodies. These Ultraviolet light (there are different forms of this by visual recognition, though a often may tests are not specific for localized scleroderma and therapy, depending on the light wavelength being be done to show increased deposition and may be abnormal in other conditions, including used) has also shown considerable promise in patients Funding for this brochure was provided by an unrestricted other suggestive changes upon microscopic analysis the internal type of scleroderma. However, with active disease. In other situations or when educational grant from Pharmaceuticals USA, Inc. of the skin. The biopsy may also help determine specific antibodies that are common in systemic these treatments fail, there are other medicines that whether the disease is still active. However, skin sclerosis (typically anti-centromere antibodies or can be tried in spite of the fact that they have not lesions may extend even when the biopsy does not anti- or SCL70) are expected to be been proven to work yet in careful clinical research show any significant changes. The process of taking negative in patients with localized scleroderma. studies. There is definite agreement that physical a involves first numbing the skin with therapy to preserve full motion of the affected joints an injection containing a numbing (for What Medicines Are is important. example, lidocaine), and then taking a small (3-4 May 2015 mm or about 0.15 inches) circular area of skin with Available? Please note that this brochure is provided for a special “punch biopsy” device. Generally, unless There is no absolute cure for localized scleroderma. educational purposed only. It is not intended to one is dealing with a much deeper involvement of An absolute cure could be defined as not only substitute for informed medical advice. the skin, larger are not necessary. Stitches complete stabilization of the skin lesions, but also are optional when the biopsy site is very small. Any disappearance of the changes that have taken place in The Scleroderma Foundation wishes to thank Vincent Falanga, biopsy will result in a small . the skin, under the skin, and any associated cosmetic M.D., Boston University, for his contribution to this brochure. Introduction be an but, other than the presence a combination of different types of skin involvement. Prognosis: What Will generalized morphea. Extensive lesions of linear of blood (confusingly similar to those Patients with generalized morphea, because of the scleroderma, when cross joint lines, can impair Scleroderma means “hard skin.” The term “localized with some internal diseases), patients have no other extensive surface area involved, may encounter Happen? motion of that particular joint. Unless continued scleroderma” as a primary diagnosis refers to the fact known or profound defect in the . considerable cosmetic disability resulting from the Morphea occurs in all age groups, and tends to be efforts are made to maintain a full range of motion Become a Member of the that this group of conditions is “localized” to the skin, appearance of the problem (many dark and light areas more common in women. to the affected joint with , this Scleroderma Foundation with no internal organ involvement. Sometimes, of skin). Also, because of skin thickening over the Most patients develop only one or two patches of complication may be permanent and result in the When you become a the term “morphea” is used interchangeably with Types of Localized The Member Magazine of the Scleroderma Foundation joints, patients may have limited joint function. thickening that are frequently darker or lighter affected area (for example, the elbow, arm, finger, member of the localized scleroderma, creating some confusion. Scleroderma Scleroderma than the surrounding skin. A yellow discoloration etc.) being in a fixed position (contracture). Many Scleroderma Foundation, Morphea, in fact, is only one particular type of C) Linear Scleroderma – Linear scleroderma, patients with linear scleroderma, especially if older VOICE Names and terminology are widely varied and cause Rewind also may occur. The changes in skin color may last you support the localized scleroderma. It is important to understand as the name implies, shows a band or line of skin San Francisco a great deal of confusion in localized scleroderma. for years, in spite of improvement and softening at the age of onset of the disease, will have only organization’s mission of National Patient that localized scleroderma is different from the Patients are often told they have “scleroderma,” which thickening. It may extend deep into the skin and minor skin changes and minimal skin thickening. Education Conference even involve the underlying muscle. The band of skin of the skin. In general, morphea tends to involve support, education and form of scleroderma which affects internal organs, may frighten them. A newly diagnosed patient may Linear scleroderma remains active for two to five Challenges for Kids thickening is more common on the legs and arms and, the superficial layers of the skin. Patients with research. Your donation with Scleroderma called systemic sclerosis or, often incorrectly stated, think they have systemic sclerosis and will develop morphea do quite well, but periodic follow-up years, but can last longer in some cases. Sometimes as . Localized scleroderma when crossing the joints, may prevent proper joint helps pay for programs internal organ involvement. This is not true. is recommended. Rarely, patients will continue patients develop recurrences after a period of what Ernie Ross typically only affects the skin, although in some cases motion. On rare occasions linear scleroderma can was thought to be inactive disease. This “recurrence” in each of those three Racing to Victory There are four main types of localized scleroderma. to develop new spots and essentially develop into areas, including: be a serious problem in children, especially when it Fall 2011 the underlying muscle and may be involved generalized morphea. This evolution to generalized is more frequent in patients with “en coup de sabre.” www.scleroderma.org (subcutaneous morphea). Localized scleroderma is Each type is characterized by the shape and amount extends deep into the skin. Sometimes, for reasons of affected skin. The four types are: A) morphea B) morphea is more common in women. En coup de sabre is potentially the most disfiguring • We budget at least $1 million annually not a fatal disease, but quality of life is often adversely we do not yet understand, linear scleroderma delays generalized morphea C) linear scleroderma and D) en form of localized scleroderma, because it affects affected because of changes in the appearance of the growth of the underlying in children who are Generalized morphea occurs in all age groups, for research. coup de sabre. the face and . It can be mild, with only slight skin, the occurrence of joint contractures that affect still in an active growth phase. and patients with this type are expected to have more extensive and prolonged periods when the of the skin. However, depending on its • Helping patients and their families cope with movement, and, rarely, serious deformities of the face A) Morphea – This is the most common type D) En Coup de Sabre – An unusual form of disease is active, in most cases lasting several years. locations on the face, it can lead to considerable scleroderma through mutual support groups, and extremities. of localized scleroderma. It presents as one or a linear scleroderma on the face or scalp may appear Generalized morphea represents more than just a problems, especially in children. It is possible that referrals and the National Patient The goal of this pamphlet is to inform you about few (3-4) patches of skin thickening with different as a white line referred to as “en coup de sabre.” greater number of morphea lesions, but rather a it is an entity by itself, and not truly a type of linear Education Conference. the nature, progress and complications of localized degrees of pigment changes. Some areas are dark This is a French term meaning “cut from a sword,” greater tendency for the lesions to merge together. scleroderma. If located on the scalp, it can cause scleroderma, and to discuss current treatment options. while others are lighter than the surrounding normal because of the way it looks. Some people think it may The areas of skin thickening in generalized morphea varied degrees of . When involving the face, • Promoting public education of the disease Because localized scleroderma often affects children, skin. Often, the skin is not quite hard to the be completely different from linear scleroderma. tend to become confluent (joined together), it can lead to indentations or depressions of the skin through publications, seminars, patient a major goal of the pamphlet is to also alleviate fears touch. It is generally painless, but pruritus (itching) En coup de sabre can be very destructive, as when occasionally involving most of the body surface, and surface, especially on the forehead. The process can education events and awareness activities. extend to the underlying . Recurrences can about this condition and to explain how it differs from may be present. A violet-colored border may be seen it results in atrophy (loss of tissue) of the face in the depth of involvement is generally greater than in when the lesions are still very active and extending. children; this process may involve the tongue and morphea. Periodic follow-up is recommended. occur, even when it seems the disease has gone into As a member of the Scleroderma systemic sclerosis, which is quite different and affects . internal organs and the skin. Sometimes, doctors will classify morphea further into mouth. Rarely, the condition is associated with Foundation, you will receive: other sub-types, according to the shape or depth of abnormalities in the growth of facial bones, which can Depending on the degree of skin involvement, patients with generalized morphea may have severe the lesions. For example, “guttate” morphea refers to potentially lead to considerable deformities. There Does Localized • Our quarterly magazine, the “Scleroderma What Is Localized “drop-like” shaped areas of skin involvement, whereas may be some overlap between en coup de sabre and from the changes caused by extensive skin thickening. Eventually, as with morphea, Scleroderma Go Away? VOICE.” The magazine includes updates on Scleroderma? “subcutaneous” morphea indicates a substantial a rare atrophy in the face, known as Parry-Romberg As a general rule, localized scleroderma is a self- the latest scleroderma research and treatments, involvement of deeper tissues with relative sparing of syndrome. softening of the skin is expected, but the skin Localized scleroderma is characterized by discoloration may last for many years and could limiting problem, at least in terms of activity of positive and uplifting stories from patients living the overlying skin. The subcutaneous type may extend and thickening of the skin from excessive remain permanent. This is because the increased the process; the color changes are likely to remain. with the disease; and tips about how to manage deep into muscle tissues in very rare instances, but this collagen deposition. Collagen is a normally How Is Localized pigment persists in the deeper portions of the skin Sometimes, new lesions may appear for a few years, living with scleroderma. does not indicate internal organ involvement. present in our skin. It provides structural support. Scleroderma Diagnosed? and cannot be removed easily by bleaching agents. but eventually, the process of developing new areas of involvement will subside. The one possible exception • Information and educational offerings from your However, when too much collagen is made, the skin B) Generalized Morphea – Generalized Doctors who are familiar with scleroderma, or who Linear scleroderma is more common in children becomes stiff and hard. to this statement is en coup de sabre, which may run local chapter. morphea involves larger skin patches than morphea, are experts at examining the skin, can arrive at the and adolescents. About 80 percent of patients an unpredictable course and become active again, often including more of the body surface. Rarely, diagnosis without much difficulty after a careful diagnosed with linear scleroderma are younger than even many years or decades after it first appeared. • Discounted registration fees to the annual What Causes Localized most of the body may be involved. Some patients with examination. In some cases, further tests may be 20. Women are affected about four times more than National Patient Education Conference. generalized morphea also have a band of thickening on needed to confirm the diagnosis. These tests may men. Linear scleroderma has the potential to cause Scleroderma? Are There Other an arm or leg as seen in linear scleroderma, another include taking a small sample of the skin (a biopsy) serious complications. The linear areas of the skin Please consider joining the Scleroderma The cause is unknown. It is not infectious and cannot type of localized scleroderma (see below). Moreover, and some blood samples. It’s important that the entire thickening may extend to the underlying tissue and Complications of Localized Scleroderma? Foundation today. A membership form is be spread by touch or contact with the patient. It individual patches of morphea are common in linear skin surface be examined, so that a complete record muscle in children, which may impair growth in attached on the reverse side of this panel. is not hereditary; however, in rare instances similar scleroderma. Therefore, although one type of localized is made of what is present at first (baseline record). the affected leg or arm. The surface changes in the Some patients with localized scleroderma, an problems may be present in relatives. It is thought to scleroderma usually predominates, patients may have Photographic documentation is also valuable. skin are similar to those described for morphea and estimated 10 to 20 percent, develop joint pain Introduction be an autoimmune disease but, other than the presence a combination of different types of skin involvement. Prognosis: What Will generalized morphea. Extensive lesions of linear of blood autoantibodies (confusingly similar to those Patients with generalized morphea, because of the scleroderma, when cross joint lines, can impair Scleroderma means “hard skin.” The term “localized with some internal diseases), patients have no other extensive surface area involved, may encounter Happen? motion of that particular joint. Unless continued scleroderma” as a primary diagnosis refers to the fact known or profound defect in the immune system. considerable cosmetic disability resulting from the Morphea occurs in all age groups, and tends to be efforts are made to maintain a full range of motion Become a Member of the that this group of conditions is “localized” to the skin, appearance of the problem (many dark and light areas more common in women. to the affected joint with physical therapy, this Scleroderma Foundation with no internal organ involvement. Sometimes, of skin). Also, because of skin thickening over the Most patients develop only one or two patches of complication may be permanent and result in the When you become a the term “morphea” is used interchangeably with Types of Localized The Member Magazine of the Scleroderma Foundation joints, patients may have limited joint function. thickening that are frequently darker or lighter affected area (for example, the elbow, arm, finger, member of the localized scleroderma, creating some confusion. Scleroderma Scleroderma than the surrounding skin. A yellow discoloration etc.) being in a fixed position (contracture). Many Scleroderma Foundation, Morphea, in fact, is only one particular type of C) Linear Scleroderma – Linear scleroderma, patients with linear scleroderma, especially if older VOICE Names and terminology are widely varied and cause Rewind also may occur. The changes in skin color may last you support the localized scleroderma. It is important to understand as the name implies, shows a band or line of skin San Francisco a great deal of confusion in localized scleroderma. for years, in spite of improvement and softening at the age of onset of the disease, will have only organization’s mission of National Patient that localized scleroderma is different from the Patients are often told they have “scleroderma,” which thickening. It may extend deep into the skin and minor skin changes and minimal skin thickening. Education Conference even involve the underlying muscle. The band of skin of the skin. In general, morphea tends to involve support, education and form of scleroderma which affects internal organs, may frighten them. A newly diagnosed patient may Linear scleroderma remains active for two to five Challenges for Kids thickening is more common on the legs and arms and, the superficial layers of the skin. Patients with research. Your donation with Scleroderma called systemic sclerosis or, often incorrectly stated, think they have systemic sclerosis and will develop morphea do quite well, but periodic follow-up years, but can last longer in some cases. Sometimes as systemic scleroderma. Localized scleroderma when crossing the joints, may prevent proper joint helps pay for programs internal organ involvement. This is not true. is recommended. Rarely, patients will continue patients develop recurrences after a period of what Ernie Ross typically only affects the skin, although in some cases motion. On rare occasions linear scleroderma can was thought to be inactive disease. This “recurrence” in each of those three Racing to Victory There are four main types of localized scleroderma. to develop new spots and essentially develop into areas, including: be a serious problem in children, especially when it Fall 2011 the underlying muscle and tissue may be involved generalized morphea. This evolution to generalized is more frequent in patients with “en coup de sabre.” www.scleroderma.org (subcutaneous morphea). Localized scleroderma is Each type is characterized by the shape and amount extends deep into the skin. Sometimes, for reasons of affected skin. The four types are: A) morphea B) morphea is more common in women. En coup de sabre is potentially the most disfiguring • We budget at least $1 million annually not a fatal disease, but quality of life is often adversely we do not yet understand, linear scleroderma delays generalized morphea C) linear scleroderma and D) en form of localized scleroderma, because it affects affected because of changes in the appearance of the growth of the underlying bones in children who are Generalized morphea occurs in all age groups, for research. coup de sabre. the face and scalp. It can be mild, with only slight skin, the occurrence of joint contractures that affect still in an active growth phase. and patients with this type are expected to have more extensive and prolonged periods when the atrophy of the skin. However, depending on its • Helping patients and their families cope with movement, and, rarely, serious deformities of the face A) Morphea – This is the most common type D) En Coup de Sabre – An unusual form of disease is active, in most cases lasting several years. locations on the face, it can lead to considerable scleroderma through mutual support groups, and extremities. of localized scleroderma. It presents as one or a linear scleroderma on the face or scalp may appear Generalized morphea represents more than just a problems, especially in children. It is possible that physician referrals and the National Patient The goal of this pamphlet is to inform you about few (3-4) patches of skin thickening with different as a white line referred to as “en coup de sabre.” greater number of morphea lesions, but rather a it is an entity by itself, and not truly a type of linear Education Conference. the nature, progress and complications of localized degrees of pigment changes. Some areas are dark This is a French term meaning “cut from a sword,” greater tendency for the lesions to merge together. scleroderma. If located on the scalp, it can cause scleroderma, and to discuss current treatment options. while others are lighter than the surrounding normal because of the way it looks. Some people think it may The areas of skin thickening in generalized morphea varied degrees of hair loss. When involving the face, • Promoting public education of the disease Because localized scleroderma often affects children, skin. Often, the skin lesion is not quite hard to the be completely different from linear scleroderma. tend to become confluent (joined together), it can lead to indentations or depressions of the skin through publications, seminars, patient a major goal of the pamphlet is to also alleviate fears touch. It is generally painless, but pruritus (itching) En coup de sabre can be very destructive, as when occasionally involving most of the body surface, and surface, especially on the forehead. The process can education events and awareness activities. extend to the underlying bone. Recurrences can about this condition and to explain how it differs from may be present. A violet-colored border may be seen it results in atrophy (loss of tissue) of the face in the depth of involvement is generally greater than in when the lesions are still very active and extending. children; this process may involve the tongue and morphea. Periodic follow-up is recommended. occur, even when it seems the disease has gone into As a member of the Scleroderma systemic sclerosis, which is quite different and affects remission. internal organs and the skin. Sometimes, doctors will classify morphea further into mouth. Rarely, the condition is associated with Foundation, you will receive: other sub-types, according to the shape or depth of abnormalities in the growth of facial bones, which can Depending on the degree of skin involvement, patients with generalized morphea may have severe the lesions. For example, “guttate” morphea refers to potentially lead to considerable deformities. There Does Localized • Our quarterly magazine, the “Scleroderma What Is Localized “drop-like” shaped areas of skin involvement, whereas may be some overlap between en coup de sabre and disfigurement from the changes caused by extensive skin thickening. Eventually, as with morphea, Scleroderma Go Away? VOICE.” The magazine includes updates on Scleroderma? “subcutaneous” morphea indicates a substantial a rare atrophy in the face, known as Parry-Romberg As a general rule, localized scleroderma is a self- the latest scleroderma research and treatments, involvement of deeper tissues with relative sparing of syndrome. softening of the skin is expected, but the skin Localized scleroderma is characterized by discoloration may last for many years and could limiting problem, at least in terms of activity of positive and uplifting stories from patients living the overlying skin. The subcutaneous type may extend inflammation and thickening of the skin from excessive remain permanent. This is because the increased the process; the color changes are likely to remain. with the disease; and tips about how to manage deep into muscle tissues in very rare instances, but this collagen deposition. Collagen is a protein normally How Is Localized pigment persists in the deeper portions of the skin Sometimes, new lesions may appear for a few years, living with scleroderma. does not indicate internal organ involvement. present in our skin. It provides structural support. Scleroderma Diagnosed? and cannot be removed easily by bleaching agents. but eventually, the process of developing new areas of involvement will subside. The one possible exception • Information and educational offerings from your However, when too much collagen is made, the skin B) Generalized Morphea – Generalized Doctors who are familiar with scleroderma, or who Linear scleroderma is more common in children becomes stiff and hard. to this statement is en coup de sabre, which may run local chapter. morphea involves larger skin patches than morphea, are experts at examining the skin, can arrive at the and adolescents. About 80 percent of patients an unpredictable course and become active again, often including more of the body surface. Rarely, diagnosis without much difficulty after a careful diagnosed with linear scleroderma are younger than even many years or decades after it first appeared. • Discounted registration fees to the annual What Causes Localized most of the body may be involved. Some patients with examination. In some cases, further tests may be 20. Women are affected about four times more than National Patient Education Conference. generalized morphea also have a band of thickening on needed to confirm the diagnosis. These tests may men. Linear scleroderma has the potential to cause Scleroderma? Are There Other an arm or leg as seen in linear scleroderma, another include taking a small sample of the skin (a biopsy) serious complications. The linear areas of the skin Please consider joining the Scleroderma The cause is unknown. It is not infectious and cannot type of localized scleroderma (see below). Moreover, and some blood samples. It’s important that the entire thickening may extend to the underlying tissue and Complications of Localized Scleroderma? Foundation today. A membership form is be spread by touch or contact with the patient. It individual patches of morphea are common in linear skin surface be examined, so that a complete record muscle in children, which may impair growth in attached on the reverse side of this panel. is not hereditary; however, in rare instances similar scleroderma. Therefore, although one type of localized is made of what is present at first (baseline record). the affected leg or arm. The surface changes in the Some patients with localized scleroderma, an problems may be present in relatives. It is thought to scleroderma usually predominates, patients may have Photographic documentation is also valuable. skin are similar to those described for morphea and estimated 10 to 20 percent, develop joint pain Introduction be an autoimmune disease but, other than the presence a combination of different types of skin involvement. Prognosis: What Will generalized morphea. Extensive lesions of linear of blood autoantibodies (confusingly similar to those Patients with generalized morphea, because of the scleroderma, when cross joint lines, can impair Scleroderma means “hard skin.” The term “localized with some internal diseases), patients have no other extensive surface area involved, may encounter Happen? motion of that particular joint. Unless continued scleroderma” as a primary diagnosis refers to the fact known or profound defect in the immune system. considerable cosmetic disability resulting from the Morphea occurs in all age groups, and tends to be efforts are made to maintain a full range of motion Become a Member of the that this group of conditions is “localized” to the skin, appearance of the problem (many dark and light areas more common in women. to the affected joint with physical therapy, this Scleroderma Foundation with no internal organ involvement. Sometimes, of skin). Also, because of skin thickening over the Most patients develop only one or two patches of complication may be permanent and result in the When you become a the term “morphea” is used interchangeably with Types of Localized The Member Magazine of the Scleroderma Foundation joints, patients may have limited joint function. thickening that are frequently darker or lighter affected area (for example, the elbow, arm, finger, member of the localized scleroderma, creating some confusion. Scleroderma Scleroderma than the surrounding skin. A yellow discoloration etc.) being in a fixed position (contracture). Many Scleroderma Foundation, Morphea, in fact, is only one particular type of C) Linear Scleroderma – Linear scleroderma, patients with linear scleroderma, especially if older VOICE Names and terminology are widely varied and cause Rewind also may occur. The changes in skin color may last you support the localized scleroderma. It is important to understand as the name implies, shows a band or line of skin San Francisco a great deal of confusion in localized scleroderma. for years, in spite of improvement and softening at the age of onset of the disease, will have only organization’s mission of National Patient that localized scleroderma is different from the Patients are often told they have “scleroderma,” which thickening. It may extend deep into the skin and minor skin changes and minimal skin thickening. Education Conference even involve the underlying muscle. The band of skin of the skin. In general, morphea tends to involve support, education and form of scleroderma which affects internal organs, may frighten them. A newly diagnosed patient may Linear scleroderma remains active for two to five Challenges for Kids thickening is more common on the legs and arms and, the superficial layers of the skin. Patients with research. Your donation with Scleroderma called systemic sclerosis or, often incorrectly stated, think they have systemic sclerosis and will develop morphea do quite well, but periodic follow-up years, but can last longer in some cases. Sometimes as systemic scleroderma. Localized scleroderma when crossing the joints, may prevent proper joint helps pay for programs internal organ involvement. This is not true. is recommended. Rarely, patients will continue patients develop recurrences after a period of what Ernie Ross typically only affects the skin, although in some cases motion. On rare occasions linear scleroderma can was thought to be inactive disease. This “recurrence” in each of those three Racing to Victory There are four main types of localized scleroderma. to develop new spots and essentially develop into areas, including: be a serious problem in children, especially when it Fall 2011 the underlying muscle and tissue may be involved generalized morphea. This evolution to generalized is more frequent in patients with “en coup de sabre.” www.scleroderma.org (subcutaneous morphea). Localized scleroderma is Each type is characterized by the shape and amount extends deep into the skin. Sometimes, for reasons of affected skin. The four types are: A) morphea B) morphea is more common in women. En coup de sabre is potentially the most disfiguring • We budget at least $1 million annually not a fatal disease, but quality of life is often adversely we do not yet understand, linear scleroderma delays generalized morphea C) linear scleroderma and D) en form of localized scleroderma, because it affects affected because of changes in the appearance of the growth of the underlying bones in children who are Generalized morphea occurs in all age groups, for research. coup de sabre. the face and scalp. It can be mild, with only slight skin, the occurrence of joint contractures that affect still in an active growth phase. and patients with this type are expected to have more extensive and prolonged periods when the atrophy of the skin. However, depending on its • Helping patients and their families cope with movement, and, rarely, serious deformities of the face A) Morphea – This is the most common type D) En Coup de Sabre – An unusual form of disease is active, in most cases lasting several years. locations on the face, it can lead to considerable scleroderma through mutual support groups, and extremities. of localized scleroderma. It presents as one or a linear scleroderma on the face or scalp may appear Generalized morphea represents more than just a problems, especially in children. It is possible that physician referrals and the National Patient The goal of this pamphlet is to inform you about few (3-4) patches of skin thickening with different as a white line referred to as “en coup de sabre.” greater number of morphea lesions, but rather a it is an entity by itself, and not truly a type of linear Education Conference. the nature, progress and complications of localized degrees of pigment changes. Some areas are dark This is a French term meaning “cut from a sword,” greater tendency for the lesions to merge together. scleroderma. If located on the scalp, it can cause scleroderma, and to discuss current treatment options. while others are lighter than the surrounding normal because of the way it looks. Some people think it may The areas of skin thickening in generalized morphea varied degrees of hair loss. When involving the face, • Promoting public education of the disease Because localized scleroderma often affects children, skin. Often, the skin lesion is not quite hard to the be completely different from linear scleroderma. tend to become confluent (joined together), it can lead to indentations or depressions of the skin through publications, seminars, patient a major goal of the pamphlet is to also alleviate fears touch. It is generally painless, but pruritus (itching) En coup de sabre can be very destructive, as when occasionally involving most of the body surface, and surface, especially on the forehead. The process can education events and awareness activities. extend to the underlying bone. Recurrences can about this condition and to explain how it differs from may be present. A violet-colored border may be seen it results in atrophy (loss of tissue) of the face in the depth of involvement is generally greater than in when the lesions are still very active and extending. children; this process may involve the tongue and morphea. Periodic follow-up is recommended. occur, even when it seems the disease has gone into As a member of the Scleroderma systemic sclerosis, which is quite different and affects remission. internal organs and the skin. Sometimes, doctors will classify morphea further into mouth. Rarely, the condition is associated with Foundation, you will receive: other sub-types, according to the shape or depth of abnormalities in the growth of facial bones, which can Depending on the degree of skin involvement, patients with generalized morphea may have severe the lesions. For example, “guttate” morphea refers to potentially lead to considerable deformities. There Does Localized • Our quarterly magazine, the “Scleroderma What Is Localized “drop-like” shaped areas of skin involvement, whereas may be some overlap between en coup de sabre and disfigurement from the changes caused by extensive skin thickening. Eventually, as with morphea, Scleroderma Go Away? VOICE.” The magazine includes updates on Scleroderma? “subcutaneous” morphea indicates a substantial a rare atrophy in the face, known as Parry-Romberg As a general rule, localized scleroderma is a self- the latest scleroderma research and treatments, involvement of deeper tissues with relative sparing of syndrome. softening of the skin is expected, but the skin Localized scleroderma is characterized by discoloration may last for many years and could limiting problem, at least in terms of activity of positive and uplifting stories from patients living the overlying skin. The subcutaneous type may extend inflammation and thickening of the skin from excessive remain permanent. This is because the increased the process; the color changes are likely to remain. with the disease; and tips about how to manage deep into muscle tissues in very rare instances, but this collagen deposition. Collagen is a protein normally How Is Localized pigment persists in the deeper portions of the skin Sometimes, new lesions may appear for a few years, living with scleroderma. does not indicate internal organ involvement. present in our skin. It provides structural support. Scleroderma Diagnosed? and cannot be removed easily by bleaching agents. but eventually, the process of developing new areas of involvement will subside. The one possible exception • Information and educational offerings from your However, when too much collagen is made, the skin B) Generalized Morphea – Generalized Doctors who are familiar with scleroderma, or who Linear scleroderma is more common in children becomes stiff and hard. to this statement is en coup de sabre, which may run local chapter. morphea involves larger skin patches than morphea, are experts at examining the skin, can arrive at the and adolescents. About 80 percent of patients an unpredictable course and become active again, often including more of the body surface. Rarely, diagnosis without much difficulty after a careful diagnosed with linear scleroderma are younger than even many years or decades after it first appeared. • Discounted registration fees to the annual What Causes Localized most of the body may be involved. Some patients with examination. In some cases, further tests may be 20. Women are affected about four times more than National Patient Education Conference. generalized morphea also have a band of thickening on needed to confirm the diagnosis. These tests may men. Linear scleroderma has the potential to cause Scleroderma? Are There Other an arm or leg as seen in linear scleroderma, another include taking a small sample of the skin (a biopsy) serious complications. The linear areas of the skin Please consider joining the Scleroderma The cause is unknown. It is not infectious and cannot type of localized scleroderma (see below). Moreover, and some blood samples. It’s important that the entire thickening may extend to the underlying tissue and Complications of Localized Scleroderma? Foundation today. A membership form is be spread by touch or contact with the patient. It individual patches of morphea are common in linear skin surface be examined, so that a complete record muscle in children, which may impair growth in attached on the reverse side of this panel. is not hereditary; however, in rare instances similar scleroderma. Therefore, although one type of localized is made of what is present at first (baseline record). the affected leg or arm. The surface changes in the Some patients with localized scleroderma, an problems may be present in relatives. It is thought to scleroderma usually predominates, patients may have Photographic documentation is also valuable. skin are similar to those described for morphea and estimated 10 to 20 percent, develop joint pain Introduction be an autoimmune disease but, other than the presence a combination of different types of skin involvement. Prognosis: What Will generalized morphea. Extensive lesions of linear of blood autoantibodies (confusingly similar to those Patients with generalized morphea, because of the scleroderma, when cross joint lines, can impair Scleroderma means “hard skin.” The term “localized with some internal diseases), patients have no other extensive surface area involved, may encounter Happen? motion of that particular joint. Unless continued scleroderma” as a primary diagnosis refers to the fact known or profound defect in the immune system. considerable cosmetic disability resulting from the Morphea occurs in all age groups, and tends to be efforts are made to maintain a full range of motion Become a Member of the that this group of conditions is “localized” to the skin, appearance of the problem (many dark and light areas more common in women. to the affected joint with physical therapy, this Scleroderma Foundation with no internal organ involvement. Sometimes, of skin). Also, because of skin thickening over the Most patients develop only one or two patches of complication may be permanent and result in the When you become a the term “morphea” is used interchangeably with Types of Localized The Member Magazine of the Scleroderma Foundation joints, patients may have limited joint function. thickening that are frequently darker or lighter affected area (for example, the elbow, arm, finger, member of the localized scleroderma, creating some confusion. Scleroderma Scleroderma than the surrounding skin. A yellow discoloration etc.) being in a fixed position (contracture). Many Scleroderma Foundation, Morphea, in fact, is only one particular type of C) Linear Scleroderma – Linear scleroderma, patients with linear scleroderma, especially if older VOICE Names and terminology are widely varied and cause Rewind also may occur. The changes in skin color may last you support the localized scleroderma. It is important to understand as the name implies, shows a band or line of skin San Francisco a great deal of confusion in localized scleroderma. for years, in spite of improvement and softening at the age of onset of the disease, will have only organization’s mission of National Patient that localized scleroderma is different from the Patients are often told they have “scleroderma,” which thickening. It may extend deep into the skin and minor skin changes and minimal skin thickening. Education Conference even involve the underlying muscle. The band of skin of the skin. In general, morphea tends to involve support, education and form of scleroderma which affects internal organs, may frighten them. A newly diagnosed patient may Linear scleroderma remains active for two to five Challenges for Kids thickening is more common on the legs and arms and, the superficial layers of the skin. Patients with research. Your donation with Scleroderma called systemic sclerosis or, often incorrectly stated, think they have systemic sclerosis and will develop morphea do quite well, but periodic follow-up years, but can last longer in some cases. Sometimes as systemic scleroderma. Localized scleroderma when crossing the joints, may prevent proper joint helps pay for programs internal organ involvement. This is not true. is recommended. Rarely, patients will continue patients develop recurrences after a period of what Ernie Ross typically only affects the skin, although in some cases motion. On rare occasions linear scleroderma can was thought to be inactive disease. This “recurrence” in each of those three Racing to Victory There are four main types of localized scleroderma. to develop new spots and essentially develop into areas, including: be a serious problem in children, especially when it Fall 2011 the underlying muscle and tissue may be involved generalized morphea. This evolution to generalized is more frequent in patients with “en coup de sabre.” www.scleroderma.org (subcutaneous morphea). Localized scleroderma is Each type is characterized by the shape and amount extends deep into the skin. Sometimes, for reasons of affected skin. The four types are: A) morphea B) morphea is more common in women. En coup de sabre is potentially the most disfiguring • We budget at least $1 million annually not a fatal disease, but quality of life is often adversely we do not yet understand, linear scleroderma delays generalized morphea C) linear scleroderma and D) en form of localized scleroderma, because it affects affected because of changes in the appearance of the growth of the underlying bones in children who are Generalized morphea occurs in all age groups, for research. coup de sabre. the face and scalp. It can be mild, with only slight skin, the occurrence of joint contractures that affect still in an active growth phase. and patients with this type are expected to have more extensive and prolonged periods when the atrophy of the skin. However, depending on its • Helping patients and their families cope with movement, and, rarely, serious deformities of the face A) Morphea – This is the most common type D) En Coup de Sabre – An unusual form of disease is active, in most cases lasting several years. locations on the face, it can lead to considerable scleroderma through mutual support groups, and extremities. of localized scleroderma. It presents as one or a linear scleroderma on the face or scalp may appear Generalized morphea represents more than just a problems, especially in children. It is possible that physician referrals and the National Patient The goal of this pamphlet is to inform you about few (3-4) patches of skin thickening with different as a white line referred to as “en coup de sabre.” greater number of morphea lesions, but rather a it is an entity by itself, and not truly a type of linear Education Conference. the nature, progress and complications of localized degrees of pigment changes. Some areas are dark This is a French term meaning “cut from a sword,” greater tendency for the lesions to merge together. scleroderma. If located on the scalp, it can cause scleroderma, and to discuss current treatment options. while others are lighter than the surrounding normal because of the way it looks. Some people think it may The areas of skin thickening in generalized morphea varied degrees of hair loss. When involving the face, • Promoting public education of the disease Because localized scleroderma often affects children, skin. Often, the skin lesion is not quite hard to the be completely different from linear scleroderma. tend to become confluent (joined together), it can lead to indentations or depressions of the skin through publications, seminars, patient a major goal of the pamphlet is to also alleviate fears touch. It is generally painless, but pruritus (itching) En coup de sabre can be very destructive, as when occasionally involving most of the body surface, and surface, especially on the forehead. The process can education events and awareness activities. extend to the underlying bone. Recurrences can about this condition and to explain how it differs from may be present. A violet-colored border may be seen it results in atrophy (loss of tissue) of the face in the depth of involvement is generally greater than in when the lesions are still very active and extending. children; this process may involve the tongue and morphea. Periodic follow-up is recommended. occur, even when it seems the disease has gone into As a member of the Scleroderma systemic sclerosis, which is quite different and affects remission. internal organs and the skin. Sometimes, doctors will classify morphea further into mouth. Rarely, the condition is associated with Foundation, you will receive: other sub-types, according to the shape or depth of abnormalities in the growth of facial bones, which can Depending on the degree of skin involvement, patients with generalized morphea may have severe the lesions. For example, “guttate” morphea refers to potentially lead to considerable deformities. There Does Localized • Our quarterly magazine, the “Scleroderma What Is Localized “drop-like” shaped areas of skin involvement, whereas may be some overlap between en coup de sabre and disfigurement from the changes caused by extensive skin thickening. Eventually, as with morphea, Scleroderma Go Away? VOICE.” The magazine includes updates on Scleroderma? “subcutaneous” morphea indicates a substantial a rare atrophy in the face, known as Parry-Romberg As a general rule, localized scleroderma is a self- the latest scleroderma research and treatments, involvement of deeper tissues with relative sparing of syndrome. softening of the skin is expected, but the skin Localized scleroderma is characterized by discoloration may last for many years and could limiting problem, at least in terms of activity of positive and uplifting stories from patients living the overlying skin. The subcutaneous type may extend inflammation and thickening of the skin from excessive remain permanent. This is because the increased the process; the color changes are likely to remain. with the disease; and tips about how to manage deep into muscle tissues in very rare instances, but this collagen deposition. Collagen is a protein normally How Is Localized pigment persists in the deeper portions of the skin Sometimes, new lesions may appear for a few years, living with scleroderma. does not indicate internal organ involvement. present in our skin. It provides structural support. Scleroderma Diagnosed? and cannot be removed easily by bleaching agents. but eventually, the process of developing new areas of involvement will subside. The one possible exception • Information and educational offerings from your However, when too much collagen is made, the skin B) Generalized Morphea – Generalized Doctors who are familiar with scleroderma, or who Linear scleroderma is more common in children becomes stiff and hard. to this statement is en coup de sabre, which may run local chapter. morphea involves larger skin patches than morphea, are experts at examining the skin, can arrive at the and adolescents. About 80 percent of patients an unpredictable course and become active again, often including more of the body surface. Rarely, diagnosis without much difficulty after a careful diagnosed with linear scleroderma are younger than even many years or decades after it first appeared. • Discounted registration fees to the annual What Causes Localized most of the body may be involved. Some patients with examination. In some cases, further tests may be 20. Women are affected about four times more than National Patient Education Conference. generalized morphea also have a band of thickening on needed to confirm the diagnosis. These tests may men. Linear scleroderma has the potential to cause Scleroderma? Are There Other an arm or leg as seen in linear scleroderma, another include taking a small sample of the skin (a biopsy) serious complications. The linear areas of the skin Please consider joining the Scleroderma The cause is unknown. It is not infectious and cannot type of localized scleroderma (see below). Moreover, and some blood samples. It’s important that the entire thickening may extend to the underlying tissue and Complications of Localized Scleroderma? Foundation today. A membership form is be spread by touch or contact with the patient. It individual patches of morphea are common in linear skin surface be examined, so that a complete record muscle in children, which may impair growth in attached on the reverse side of this panel. is not hereditary; however, in rare instances similar scleroderma. Therefore, although one type of localized is made of what is present at first (baseline record). the affected leg or arm. The surface changes in the Some patients with localized scleroderma, an problems may be present in relatives. It is thought to scleroderma usually predominates, patients may have Photographic documentation is also valuable. skin are similar to those described for morphea and estimated 10 to 20 percent, develop joint pain Introduction be an autoimmune disease but, other than the presence a combination of different types of skin involvement. Prognosis: What Will generalized morphea. Extensive lesions of linear of blood autoantibodies (confusingly similar to those Patients with generalized morphea, because of the scleroderma, when cross joint lines, can impair Scleroderma means “hard skin.” The term “localized with some internal diseases), patients have no other extensive surface area involved, may encounter Happen? motion of that particular joint. Unless continued scleroderma” as a primary diagnosis refers to the fact known or profound defect in the immune system. considerable cosmetic disability resulting from the Morphea occurs in all age groups, and tends to be efforts are made to maintain a full range of motion Become a Member of the that this group of conditions is “localized” to the skin, appearance of the problem (many dark and light areas more common in women. to the affected joint with physical therapy, this Scleroderma Foundation with no internal organ involvement. Sometimes, of skin). Also, because of skin thickening over the Most patients develop only one or two patches of complication may be permanent and result in the When you become a the term “morphea” is used interchangeably with Types of Localized The Member Magazine of the Scleroderma Foundation joints, patients may have limited joint function. thickening that are frequently darker or lighter affected area (for example, the elbow, arm, finger, member of the localized scleroderma, creating some confusion. Scleroderma Scleroderma than the surrounding skin. A yellow discoloration etc.) being in a fixed position (contracture). Many Scleroderma Foundation, Morphea, in fact, is only one particular type of C) Linear Scleroderma – Linear scleroderma, patients with linear scleroderma, especially if older VOICE Names and terminology are widely varied and cause Rewind also may occur. The changes in skin color may last you support the localized scleroderma. It is important to understand as the name implies, shows a band or line of skin San Francisco a great deal of confusion in localized scleroderma. for years, in spite of improvement and softening at the age of onset of the disease, will have only organization’s mission of National Patient that localized scleroderma is different from the Patients are often told they have “scleroderma,” which thickening. It may extend deep into the skin and minor skin changes and minimal skin thickening. Education Conference even involve the underlying muscle. The band of skin of the skin. In general, morphea tends to involve support, education and form of scleroderma which affects internal organs, may frighten them. A newly diagnosed patient may Linear scleroderma remains active for two to five Challenges for Kids thickening is more common on the legs and arms and, the superficial layers of the skin. Patients with research. Your donation with Scleroderma called systemic sclerosis or, often incorrectly stated, think they have systemic sclerosis and will develop morphea do quite well, but periodic follow-up years, but can last longer in some cases. Sometimes as systemic scleroderma. Localized scleroderma when crossing the joints, may prevent proper joint helps pay for programs internal organ involvement. This is not true. is recommended. Rarely, patients will continue patients develop recurrences after a period of what Ernie Ross typically only affects the skin, although in some cases motion. On rare occasions linear scleroderma can was thought to be inactive disease. This “recurrence” in each of those three Racing to Victory There are four main types of localized scleroderma. to develop new spots and essentially develop into areas, including: be a serious problem in children, especially when it Fall 2011 the underlying muscle and tissue may be involved generalized morphea. This evolution to generalized is more frequent in patients with “en coup de sabre.” www.scleroderma.org (subcutaneous morphea). Localized scleroderma is Each type is characterized by the shape and amount extends deep into the skin. Sometimes, for reasons of affected skin. The four types are: A) morphea B) morphea is more common in women. En coup de sabre is potentially the most disfiguring • We budget at least $1 million annually not a fatal disease, but quality of life is often adversely we do not yet understand, linear scleroderma delays generalized morphea C) linear scleroderma and D) en form of localized scleroderma, because it affects affected because of changes in the appearance of the growth of the underlying bones in children who are Generalized morphea occurs in all age groups, for research. coup de sabre. the face and scalp. It can be mild, with only slight skin, the occurrence of joint contractures that affect still in an active growth phase. and patients with this type are expected to have more extensive and prolonged periods when the atrophy of the skin. However, depending on its • Helping patients and their families cope with movement, and, rarely, serious deformities of the face A) Morphea – This is the most common type D) En Coup de Sabre – An unusual form of disease is active, in most cases lasting several years. locations on the face, it can lead to considerable scleroderma through mutual support groups, and extremities. of localized scleroderma. It presents as one or a linear scleroderma on the face or scalp may appear Generalized morphea represents more than just a problems, especially in children. It is possible that physician referrals and the National Patient The goal of this pamphlet is to inform you about few (3-4) patches of skin thickening with different as a white line referred to as “en coup de sabre.” greater number of morphea lesions, but rather a it is an entity by itself, and not truly a type of linear Education Conference. the nature, progress and complications of localized degrees of pigment changes. Some areas are dark This is a French term meaning “cut from a sword,” greater tendency for the lesions to merge together. scleroderma. If located on the scalp, it can cause scleroderma, and to discuss current treatment options. while others are lighter than the surrounding normal because of the way it looks. Some people think it may The areas of skin thickening in generalized morphea varied degrees of hair loss. When involving the face, • Promoting public education of the disease Because localized scleroderma often affects children, skin. Often, the skin lesion is not quite hard to the be completely different from linear scleroderma. tend to become confluent (joined together), it can lead to indentations or depressions of the skin through publications, seminars, patient a major goal of the pamphlet is to also alleviate fears touch. It is generally painless, but pruritus (itching) En coup de sabre can be very destructive, as when occasionally involving most of the body surface, and surface, especially on the forehead. The process can education events and awareness activities. extend to the underlying bone. Recurrences can about this condition and to explain how it differs from may be present. A violet-colored border may be seen it results in atrophy (loss of tissue) of the face in the depth of involvement is generally greater than in when the lesions are still very active and extending. children; this process may involve the tongue and morphea. Periodic follow-up is recommended. occur, even when it seems the disease has gone into As a member of the Scleroderma systemic sclerosis, which is quite different and affects remission. internal organs and the skin. Sometimes, doctors will classify morphea further into mouth. Rarely, the condition is associated with Foundation, you will receive: other sub-types, according to the shape or depth of abnormalities in the growth of facial bones, which can Depending on the degree of skin involvement, patients with generalized morphea may have severe the lesions. For example, “guttate” morphea refers to potentially lead to considerable deformities. There Does Localized • Our quarterly magazine, the “Scleroderma What Is Localized “drop-like” shaped areas of skin involvement, whereas may be some overlap between en coup de sabre and disfigurement from the changes caused by extensive skin thickening. Eventually, as with morphea, Scleroderma Go Away? VOICE.” The magazine includes updates on Scleroderma? “subcutaneous” morphea indicates a substantial a rare atrophy in the face, known as Parry-Romberg As a general rule, localized scleroderma is a self- the latest scleroderma research and treatments, involvement of deeper tissues with relative sparing of syndrome. softening of the skin is expected, but the skin Localized scleroderma is characterized by discoloration may last for many years and could limiting problem, at least in terms of activity of positive and uplifting stories from patients living the overlying skin. The subcutaneous type may extend inflammation and thickening of the skin from excessive remain permanent. This is because the increased the process; the color changes are likely to remain. with the disease; and tips about how to manage deep into muscle tissues in very rare instances, but this collagen deposition. Collagen is a protein normally How Is Localized pigment persists in the deeper portions of the skin Sometimes, new lesions may appear for a few years, living with scleroderma. does not indicate internal organ involvement. present in our skin. It provides structural support. Scleroderma Diagnosed? and cannot be removed easily by bleaching agents. but eventually, the process of developing new areas of involvement will subside. The one possible exception • Information and educational offerings from your However, when too much collagen is made, the skin B) Generalized Morphea – Generalized Doctors who are familiar with scleroderma, or who Linear scleroderma is more common in children becomes stiff and hard. to this statement is en coup de sabre, which may run local chapter. morphea involves larger skin patches than morphea, are experts at examining the skin, can arrive at the and adolescents. About 80 percent of patients an unpredictable course and become active again, often including more of the body surface. Rarely, diagnosis without much difficulty after a careful diagnosed with linear scleroderma are younger than even many years or decades after it first appeared. • Discounted registration fees to the annual What Causes Localized most of the body may be involved. Some patients with examination. In some cases, further tests may be 20. Women are affected about four times more than National Patient Education Conference. generalized morphea also have a band of thickening on needed to confirm the diagnosis. These tests may men. Linear scleroderma has the potential to cause Scleroderma? Are There Other an arm or leg as seen in linear scleroderma, another include taking a small sample of the skin (a biopsy) serious complications. The linear areas of the skin Please consider joining the Scleroderma The cause is unknown. It is not infectious and cannot type of localized scleroderma (see below). Moreover, and some blood samples. It’s important that the entire thickening may extend to the underlying tissue and Complications of Localized Scleroderma? Foundation today. A membership form is be spread by touch or contact with the patient. It individual patches of morphea are common in linear skin surface be examined, so that a complete record muscle in children, which may impair growth in attached on the reverse side of this panel. is not hereditary; however, in rare instances similar scleroderma. Therefore, although one type of localized is made of what is present at first (baseline record). the affected leg or arm. The surface changes in the Some patients with localized scleroderma, an problems may be present in relatives. It is thought to scleroderma usually predominates, patients may have Photographic documentation is also valuable. skin are similar to those described for morphea and estimated 10 to 20 percent, develop joint pain Introduction be an autoimmune disease but, other than the presence a combination of different types of skin involvement. Prognosis: What Will generalized morphea. Extensive lesions of linear of blood autoantibodies (confusingly similar to those Patients with generalized morphea, because of the scleroderma, when cross joint lines, can impair Scleroderma means “hard skin.” The term “localized with some internal diseases), patients have no other extensive surface area involved, may encounter Happen? motion of that particular joint. Unless continued scleroderma” as a primary diagnosis refers to the fact known or profound defect in the immune system. considerable cosmetic disability resulting from the Morphea occurs in all age groups, and tends to be efforts are made to maintain a full range of motion Become a Member of the that this group of conditions is “localized” to the skin, appearance of the problem (many dark and light areas more common in women. to the affected joint with physical therapy, this Scleroderma Foundation with no internal organ involvement. Sometimes, of skin). Also, because of skin thickening over the Most patients develop only one or two patches of complication may be permanent and result in the When you become a the term “morphea” is used interchangeably with Types of Localized The Member Magazine of the Scleroderma Foundation joints, patients may have limited joint function. thickening that are frequently darker or lighter affected area (for example, the elbow, arm, finger, member of the localized scleroderma, creating some confusion. Scleroderma Scleroderma than the surrounding skin. A yellow discoloration etc.) being in a fixed position (contracture). Many Scleroderma Foundation, Morphea, in fact, is only one particular type of C) Linear Scleroderma – Linear scleroderma, patients with linear scleroderma, especially if older VOICE Names and terminology are widely varied and cause Rewind also may occur. The changes in skin color may last you support the localized scleroderma. It is important to understand as the name implies, shows a band or line of skin San Francisco a great deal of confusion in localized scleroderma. for years, in spite of improvement and softening at the age of onset of the disease, will have only organization’s mission of National Patient that localized scleroderma is different from the Patients are often told they have “scleroderma,” which thickening. It may extend deep into the skin and minor skin changes and minimal skin thickening. Education Conference even involve the underlying muscle. The band of skin of the skin. In general, morphea tends to involve support, education and form of scleroderma which affects internal organs, may frighten them. A newly diagnosed patient may Linear scleroderma remains active for two to five Challenges for Kids thickening is more common on the legs and arms and, the superficial layers of the skin. Patients with research. Your donation with Scleroderma called systemic sclerosis or, often incorrectly stated, think they have systemic sclerosis and will develop morphea do quite well, but periodic follow-up years, but can last longer in some cases. Sometimes as systemic scleroderma. Localized scleroderma when crossing the joints, may prevent proper joint helps pay for programs internal organ involvement. This is not true. is recommended. Rarely, patients will continue patients develop recurrences after a period of what Ernie Ross typically only affects the skin, although in some cases motion. On rare occasions linear scleroderma can was thought to be inactive disease. This “recurrence” in each of those three Racing to Victory There are four main types of localized scleroderma. to develop new spots and essentially develop into areas, including: be a serious problem in children, especially when it Fall 2011 the underlying muscle and tissue may be involved generalized morphea. This evolution to generalized is more frequent in patients with “en coup de sabre.” www.scleroderma.org (subcutaneous morphea). Localized scleroderma is Each type is characterized by the shape and amount extends deep into the skin. Sometimes, for reasons of affected skin. The four types are: A) morphea B) morphea is more common in women. En coup de sabre is potentially the most disfiguring • We budget at least $1 million annually not a fatal disease, but quality of life is often adversely we do not yet understand, linear scleroderma delays generalized morphea C) linear scleroderma and D) en form of localized scleroderma, because it affects affected because of changes in the appearance of the growth of the underlying bones in children who are Generalized morphea occurs in all age groups, for research. coup de sabre. the face and scalp. It can be mild, with only slight skin, the occurrence of joint contractures that affect still in an active growth phase. and patients with this type are expected to have more extensive and prolonged periods when the atrophy of the skin. However, depending on its • Helping patients and their families cope with movement, and, rarely, serious deformities of the face A) Morphea – This is the most common type D) En Coup de Sabre – An unusual form of disease is active, in most cases lasting several years. locations on the face, it can lead to considerable scleroderma through mutual support groups, and extremities. of localized scleroderma. It presents as one or a linear scleroderma on the face or scalp may appear Generalized morphea represents more than just a problems, especially in children. It is possible that physician referrals and the National Patient The goal of this pamphlet is to inform you about few (3-4) patches of skin thickening with different as a white line referred to as “en coup de sabre.” greater number of morphea lesions, but rather a it is an entity by itself, and not truly a type of linear Education Conference. the nature, progress and complications of localized degrees of pigment changes. Some areas are dark This is a French term meaning “cut from a sword,” greater tendency for the lesions to merge together. scleroderma. If located on the scalp, it can cause scleroderma, and to discuss current treatment options. while others are lighter than the surrounding normal because of the way it looks. Some people think it may The areas of skin thickening in generalized morphea varied degrees of hair loss. When involving the face, • Promoting public education of the disease Because localized scleroderma often affects children, skin. Often, the skin lesion is not quite hard to the be completely different from linear scleroderma. tend to become confluent (joined together), it can lead to indentations or depressions of the skin through publications, seminars, patient a major goal of the pamphlet is to also alleviate fears touch. It is generally painless, but pruritus (itching) En coup de sabre can be very destructive, as when occasionally involving most of the body surface, and surface, especially on the forehead. The process can education events and awareness activities. extend to the underlying bone. Recurrences can about this condition and to explain how it differs from may be present. A violet-colored border may be seen it results in atrophy (loss of tissue) of the face in the depth of involvement is generally greater than in when the lesions are still very active and extending. children; this process may involve the tongue and morphea. Periodic follow-up is recommended. occur, even when it seems the disease has gone into As a member of the Scleroderma systemic sclerosis, which is quite different and affects remission. internal organs and the skin. Sometimes, doctors will classify morphea further into mouth. Rarely, the condition is associated with Foundation, you will receive: other sub-types, according to the shape or depth of abnormalities in the growth of facial bones, which can Depending on the degree of skin involvement, patients with generalized morphea may have severe the lesions. For example, “guttate” morphea refers to potentially lead to considerable deformities. There Does Localized • Our quarterly magazine, the “Scleroderma What Is Localized “drop-like” shaped areas of skin involvement, whereas may be some overlap between en coup de sabre and disfigurement from the changes caused by extensive skin thickening. Eventually, as with morphea, Scleroderma Go Away? VOICE.” The magazine includes updates on Scleroderma? “subcutaneous” morphea indicates a substantial a rare atrophy in the face, known as Parry-Romberg As a general rule, localized scleroderma is a self- the latest scleroderma research and treatments, involvement of deeper tissues with relative sparing of syndrome. softening of the skin is expected, but the skin Localized scleroderma is characterized by discoloration may last for many years and could limiting problem, at least in terms of activity of positive and uplifting stories from patients living the overlying skin. The subcutaneous type may extend inflammation and thickening of the skin from excessive remain permanent. This is because the increased the process; the color changes are likely to remain. with the disease; and tips about how to manage deep into muscle tissues in very rare instances, but this collagen deposition. Collagen is a protein normally How Is Localized pigment persists in the deeper portions of the skin Sometimes, new lesions may appear for a few years, living with scleroderma. does not indicate internal organ involvement. present in our skin. It provides structural support. Scleroderma Diagnosed? and cannot be removed easily by bleaching agents. but eventually, the process of developing new areas of involvement will subside. The one possible exception • Information and educational offerings from your However, when too much collagen is made, the skin B) Generalized Morphea – Generalized Doctors who are familiar with scleroderma, or who Linear scleroderma is more common in children becomes stiff and hard. to this statement is en coup de sabre, which may run local chapter. morphea involves larger skin patches than morphea, are experts at examining the skin, can arrive at the and adolescents. About 80 percent of patients an unpredictable course and become active again, often including more of the body surface. Rarely, diagnosis without much difficulty after a careful diagnosed with linear scleroderma are younger than even many years or decades after it first appeared. • Discounted registration fees to the annual What Causes Localized most of the body may be involved. Some patients with examination. In some cases, further tests may be 20. Women are affected about four times more than National Patient Education Conference. generalized morphea also have a band of thickening on needed to confirm the diagnosis. These tests may men. Linear scleroderma has the potential to cause Scleroderma? Are There Other an arm or leg as seen in linear scleroderma, another include taking a small sample of the skin (a biopsy) serious complications. The linear areas of the skin Please consider joining the Scleroderma The cause is unknown. It is not infectious and cannot type of localized scleroderma (see below). Moreover, and some blood samples. It’s important that the entire thickening may extend to the underlying tissue and Complications of Localized Scleroderma? Foundation today. A membership form is be spread by touch or contact with the patient. It individual patches of morphea are common in linear skin surface be examined, so that a complete record muscle in children, which may impair growth in attached on the reverse side of this panel. is not hereditary; however, in rare instances similar scleroderma. Therefore, although one type of localized is made of what is present at first (baseline record). the affected leg or arm. The surface changes in the Some patients with localized scleroderma, an problems may be present in relatives. It is thought to scleroderma usually predominates, patients may have Photographic documentation is also valuable. skin are similar to those described for morphea and estimated 10 to 20 percent, develop joint pain (arthralgia) during the course of their disease. The It’s important to look for physical signs of the or functional deformity. However, stabilization Our Three-Fold Mission Is pain is not limited to joints beneath the involved internal type of scleroderma (systemic sclerosis), (inactive disease) of localized scleroderma is indeed LOCALIZED skin, but may involve various joints, such as the such as thickening of the skin on all the fingers possible. The outlook and prognosis of localized Support, Education and knees, wrists and even the spine. (sclerodactyly), and thickening of the skin on the scleroderma is generally quite favorable. First, the Research SCLERODERMA Occasionally, this complication occurs before the face (without color changes). There are other clues disease is not fatal, does not appear to decrease appearance of lesions in the skin, causing some that can be identified during the initial examination. lifespan and, except for extremely rare situations, is confusion with rheumatoid arthritis for a short For example, patients with localized scleroderma not associated with another disease that has internal period of time. Eventually, the joint pains subside, do not have the typical changes at the base of the organ involvement. Secondly, in most instances even in the face of new skin involvement but an fingernails that are common in the internal (systemic the condition is self-limited, and this definitely association with spine arthritis has been reported. sclerosis) disease: tiny blood vessel changes that applies to individual areas of morphea. In those Along the same lines, it is highly unlikely that require a special magnifying lens for proper cases, no treatment is required or advisable, except patients with localized scleroderma progress and detection. Another important question is whether for some emollients or, in some cases, mild topical develop severe systemic (internal) conditions such patients have what is called Raynaud’s phenomenon. steroids. Therefore, systemic treatment with a as rheumatoid arthritis, or lupus erythematosus, This refers to the inability of the patient’s hands to medication or other treatment interventions (for or systemic sclerosis (the internal form of withstand cold exposure, so that the fingers may be example, ultraviolet light), are reserved for more scleroderma). Still, it is important to recognize that come pale, or even experience numbness and pain, extensive forms of localized scleroderma. There morphea and other types of localized scleroderma and/or develop a temporary bluish appearance with are many publications that reference favorable may be present in several of these systemic even mild cold temperatures, or when touching results when using certain medications, but these conditions, including systemic lupus erythematosus, a cold beverage, or getting some item from the have truly not been proven to work. Examples of systemic sclerosis, and blistering diseases. An freezer. Patients with localized scleroderma these medications include: phenytoin (Dilantin), association has been found with a parasitic disease generally do not have Raynaud’s phenomenon, systemic corticosteroids (cortisone), antimalarial (toxoplasma). While this does not imply that patients which is present in the majority of patients with drugs (plaquenil, chloroquine), cyclosporine, with localized scleroderma evolve to develop these systemic sclerosis. d-penicillamine, antibiotics (presumably to treat an conditions, it does emphasize the need for follow-up underlying Lyme disease problem.) and . and periodic evaluations, especially in patients who Several blood tests may be performed, which, when A previously used vitamin D-based therapy (with continue to have active skin problems or develop abnormal, help to determine how active the disease calcitriol) has now been proven not to work. There new issues. is and how extensive or prolonged it may become. is some recent good news for patients with extensive These blood tests include the number of blood disease who require proven medicines and other Are There Any Tests to eosinophils (cell normally present in our blood), the interventions. Methotrexate (as an anti-inflammatory Prove the Diagnosis of level of blood immune proteins (immunoglobulins), drug) has proved effective in children with active and various blood auto-antibodies: antinuclear and extensive (and deep) morphea. It has some side Localized Scleroderma? antibodies (ANA), antibodies to single-stranded effects and should only be used when truly necessary. The diagnosis of localized scleroderma is mainly DNA (ssDNA) and antihistones antibodies. These Ultraviolet light (there are different forms of this by visual recognition, though a biopsy often may tests are not specific for localized scleroderma and therapy, depending on the light wavelength being be done to show increased collagen deposition and may be abnormal in other conditions, including used) has also shown considerable promise in patients Funding for this brochure was provided by an unrestricted other suggestive changes upon microscopic analysis the internal type of scleroderma. However, with active disease. In other situations or when educational grant from Actelion Pharmaceuticals USA, Inc. of the skin. The biopsy may also help determine specific antibodies that are common in systemic these treatments fail, there are other medicines that whether the disease is still active. However, skin sclerosis (typically anti-centromere antibodies or can be tried in spite of the fact that they have not lesions may extend even when the biopsy does not anti-topoisomerase or SCL70) are expected to be been proven to work yet in careful clinical research show any significant changes. The process of taking negative in patients with localized scleroderma. studies. There is definite agreement that physical a skin biopsy involves first numbing the skin with therapy to preserve full motion of the affected joints an injection containing a numbing medicine (for What Medicines Are is important. example, lidocaine), and then taking a small (3-4 May 2015 mm or about 0.15 inches) circular area of skin with Available? Please note that this brochure is provided for a special “punch biopsy” device. Generally, unless There is no absolute cure for localized scleroderma. educational purposed only. It is not intended to one is dealing with a much deeper involvement of An absolute cure could be defined as not only substitute for informed medical advice. the skin, larger biopsies are not necessary. Stitches complete stabilization of the skin lesions, but also are optional when the biopsy site is very small. Any disappearance of the changes that have taken place in The Scleroderma Foundation wishes to thank Vincent Falanga, biopsy will result in a small scar. the skin, under the skin, and any associated cosmetic M.D., Boston University, for his contribution to this brochure. (arthralgia) during the course of their disease. The It’s important to look for physical signs of the or functional deformity. However, stabilization Our Three-Fold Mission Is pain is not limited to joints beneath the involved internal type of scleroderma (systemic sclerosis), (inactive disease) of localized scleroderma is indeed LOCALIZED skin, but may involve various joints, such as the such as thickening of the skin on all the fingers possible. The outlook and prognosis of localized Support, Education and knees, wrists and even the spine. (sclerodactyly), and thickening of the skin on the scleroderma is generally quite favorable. First, the Research SCLERODERMA Occasionally, this complication occurs before the face (without color changes). There are other clues disease is not fatal, does not appear to decrease appearance of lesions in the skin, causing some that can be identified during the initial examination. lifespan and, except for extremely rare situations, is confusion with rheumatoid arthritis for a short For example, patients with localized scleroderma not associated with another disease that has internal period of time. Eventually, the joint pains subside, do not have the typical changes at the base of the organ involvement. Secondly, in most instances even in the face of new skin involvement but an fingernails that are common in the internal (systemic the condition is self-limited, and this definitely association with spine arthritis has been reported. sclerosis) disease: tiny blood vessel changes that applies to individual areas of morphea. In those Along the same lines, it is highly unlikely that require a special magnifying lens for proper cases, no treatment is required or advisable, except patients with localized scleroderma progress and detection. Another important question is whether for some emollients or, in some cases, mild topical develop severe systemic (internal) conditions such patients have what is called Raynaud’s phenomenon. steroids. Therefore, systemic treatment with a as rheumatoid arthritis, or lupus erythematosus, This refers to the inability of the patient’s hands to medication or other treatment interventions (for or systemic sclerosis (the internal form of withstand cold exposure, so that the fingers may be example, ultraviolet light), are reserved for more scleroderma). Still, it is important to recognize that come pale, or even experience numbness and pain, extensive forms of localized scleroderma. There morphea and other types of localized scleroderma and/or develop a temporary bluish appearance with are many publications that reference favorable may be present in several of these systemic even mild cold temperatures, or when touching results when using certain medications, but these conditions, including systemic lupus erythematosus, a cold beverage, or getting some item from the have truly not been proven to work. Examples of systemic sclerosis, and blistering diseases. An freezer. Patients with localized scleroderma these medications include: phenytoin (Dilantin), association has been found with a parasitic disease generally do not have Raynaud’s phenomenon, systemic corticosteroids (cortisone), antimalarial (toxoplasma). While this does not imply that patients which is present in the majority of patients with drugs (plaquenil, chloroquine), cyclosporine, with localized scleroderma evolve to develop these systemic sclerosis. d-penicillamine, antibiotics (presumably to treat an conditions, it does emphasize the need for follow-up underlying Lyme disease problem.) and vitamin E. and periodic evaluations, especially in patients who Several blood tests may be performed, which, when A previously used vitamin D-based therapy (with continue to have active skin problems or develop abnormal, help to determine how active the disease calcitriol) has now been proven not to work. There new issues. is and how extensive or prolonged it may become. is some recent good news for patients with extensive These blood tests include the number of blood disease who require proven medicines and other Are There Any Tests to eosinophils (cell normally present in our blood), the interventions. Methotrexate (as an anti-inflammatory Prove the Diagnosis of level of blood immune proteins (immunoglobulins), drug) has proved effective in children with active and various blood auto-antibodies: antinuclear and extensive (and deep) morphea. It has some side Localized Scleroderma? antibodies (ANA), antibodies to single-stranded effects and should only be used when truly necessary. The diagnosis of localized scleroderma is mainly DNA (ssDNA) and antihistones antibodies. These Ultraviolet light (there are different forms of this by visual recognition, though a biopsy often may tests are not specific for localized scleroderma and therapy, depending on the light wavelength being be done to show increased collagen deposition and may be abnormal in other conditions, including used) has also shown considerable promise in patients Funding for this brochure was provided by an unrestricted other suggestive changes upon microscopic analysis the internal type of scleroderma. However, with active disease. In other situations or when educational grant from Actelion Pharmaceuticals USA, Inc. of the skin. The biopsy may also help determine specific antibodies that are common in systemic these treatments fail, there are other medicines that whether the disease is still active. However, skin sclerosis (typically anti-centromere antibodies or can be tried in spite of the fact that they have not lesions may extend even when the biopsy does not anti-topoisomerase or SCL70) are expected to be been proven to work yet in careful clinical research show any significant changes. The process of taking negative in patients with localized scleroderma. studies. There is definite agreement that physical a skin biopsy involves first numbing the skin with therapy to preserve full motion of the affected joints an injection containing a numbing medicine (for What Medicines Are is important. example, lidocaine), and then taking a small (3-4 May 2015 mm or about 0.15 inches) circular area of skin with Available? Please note that this brochure is provided for a special “punch biopsy” device. Generally, unless There is no absolute cure for localized scleroderma. educational purposed only. It is not intended to one is dealing with a much deeper involvement of An absolute cure could be defined as not only substitute for informed medical advice. the skin, larger biopsies are not necessary. Stitches complete stabilization of the skin lesions, but also are optional when the biopsy site is very small. Any disappearance of the changes that have taken place in The Scleroderma Foundation wishes to thank Vincent Falanga, biopsy will result in a small scar. the skin, under the skin, and any associated cosmetic M.D., Boston University, for his contribution to this brochure. (arthralgia) during the course of their disease. The It’s important to look for physical signs of the or functional deformity. However, stabilization Our Three-Fold Mission Is pain is not limited to joints beneath the involved internal type of scleroderma (systemic sclerosis), (inactive disease) of localized scleroderma is indeed LOCALIZED skin, but may involve various joints, such as the such as thickening of the skin on all the fingers possible. The outlook and prognosis of localized Support, Education and knees, wrists and even the spine. (sclerodactyly), and thickening of the skin on the scleroderma is generally quite favorable. First, the Research SCLERODERMA Occasionally, this complication occurs before the face (without color changes). There are other clues disease is not fatal, does not appear to decrease appearance of lesions in the skin, causing some that can be identified during the initial examination. lifespan and, except for extremely rare situations, is confusion with rheumatoid arthritis for a short For example, patients with localized scleroderma not associated with another disease that has internal period of time. Eventually, the joint pains subside, do not have the typical changes at the base of the organ involvement. Secondly, in most instances even in the face of new skin involvement but an fingernails that are common in the internal (systemic the condition is self-limited, and this definitely association with spine arthritis has been reported. sclerosis) disease: tiny blood vessel changes that applies to individual areas of morphea. In those Along the same lines, it is highly unlikely that require a special magnifying lens for proper cases, no treatment is required or advisable, except patients with localized scleroderma progress and detection. Another important question is whether for some emollients or, in some cases, mild topical develop severe systemic (internal) conditions such patients have what is called Raynaud’s phenomenon. steroids. Therefore, systemic treatment with a as rheumatoid arthritis, or lupus erythematosus, This refers to the inability of the patient’s hands to medication or other treatment interventions (for or systemic sclerosis (the internal form of withstand cold exposure, so that the fingers may be example, ultraviolet light), are reserved for more scleroderma). Still, it is important to recognize that come pale, or even experience numbness and pain, extensive forms of localized scleroderma. There morphea and other types of localized scleroderma and/or develop a temporary bluish appearance with are many publications that reference favorable may be present in several of these systemic even mild cold temperatures, or when touching results when using certain medications, but these conditions, including systemic lupus erythematosus, a cold beverage, or getting some item from the have truly not been proven to work. Examples of systemic sclerosis, and blistering diseases. An freezer. Patients with localized scleroderma these medications include: phenytoin (Dilantin), association has been found with a parasitic disease generally do not have Raynaud’s phenomenon, systemic corticosteroids (cortisone), antimalarial (toxoplasma). While this does not imply that patients which is present in the majority of patients with drugs (plaquenil, chloroquine), cyclosporine, with localized scleroderma evolve to develop these systemic sclerosis. d-penicillamine, antibiotics (presumably to treat an conditions, it does emphasize the need for follow-up underlying Lyme disease problem.) and vitamin E. and periodic evaluations, especially in patients who Several blood tests may be performed, which, when A previously used vitamin D-based therapy (with continue to have active skin problems or develop abnormal, help to determine how active the disease calcitriol) has now been proven not to work. There new issues. is and how extensive or prolonged it may become. is some recent good news for patients with extensive These blood tests include the number of blood disease who require proven medicines and other Are There Any Tests to eosinophils (cell normally present in our blood), the interventions. Methotrexate (as an anti-inflammatory Prove the Diagnosis of level of blood immune proteins (immunoglobulins), drug) has proved effective in children with active and various blood auto-antibodies: antinuclear and extensive (and deep) morphea. It has some side Localized Scleroderma? antibodies (ANA), antibodies to single-stranded effects and should only be used when truly necessary. The diagnosis of localized scleroderma is mainly DNA (ssDNA) and antihistones antibodies. These Ultraviolet light (there are different forms of this by visual recognition, though a biopsy often may tests are not specific for localized scleroderma and therapy, depending on the light wavelength being be done to show increased collagen deposition and may be abnormal in other conditions, including used) has also shown considerable promise in patients Funding for this brochure was provided by an unrestricted other suggestive changes upon microscopic analysis the internal type of scleroderma. However, with active disease. In other situations or when educational grant from Actelion Pharmaceuticals USA, Inc. of the skin. The biopsy may also help determine specific antibodies that are common in systemic these treatments fail, there are other medicines that whether the disease is still active. However, skin sclerosis (typically anti-centromere antibodies or can be tried in spite of the fact that they have not lesions may extend even when the biopsy does not anti-topoisomerase or SCL70) are expected to be been proven to work yet in careful clinical research show any significant changes. The process of taking negative in patients with localized scleroderma. studies. There is definite agreement that physical a skin biopsy involves first numbing the skin with therapy to preserve full motion of the affected joints an injection containing a numbing medicine (for What Medicines Are is important. example, lidocaine), and then taking a small (3-4 May 2015 mm or about 0.15 inches) circular area of skin with Available? Please note that this brochure is provided for a special “punch biopsy” device. Generally, unless There is no absolute cure for localized scleroderma. educational purposed only. It is not intended to one is dealing with a much deeper involvement of An absolute cure could be defined as not only substitute for informed medical advice. the skin, larger biopsies are not necessary. Stitches complete stabilization of the skin lesions, but also are optional when the biopsy site is very small. Any disappearance of the changes that have taken place in The Scleroderma Foundation wishes to thank Vincent Falanga, biopsy will result in a small scar. the skin, under the skin, and any associated cosmetic M.D., Boston University, for his contribution to this brochure. (arthralgia) during the course of their disease. The It’s important to look for physical signs of the or functional deformity. However, stabilization Our Three-Fold Mission Is pain is not limited to joints beneath the involved internal type of scleroderma (systemic sclerosis), (inactive disease) of localized scleroderma is indeed LOCALIZED skin, but may involve various joints, such as the such as thickening of the skin on all the fingers possible. The outlook and prognosis of localized Support, Education and knees, wrists and even the spine. (sclerodactyly), and thickening of the skin on the scleroderma is generally quite favorable. First, the Research SCLERODERMA Occasionally, this complication occurs before the face (without color changes). There are other clues disease is not fatal, does not appear to decrease appearance of lesions in the skin, causing some that can be identified during the initial examination. lifespan and, except for extremely rare situations, is confusion with rheumatoid arthritis for a short For example, patients with localized scleroderma not associated with another disease that has internal period of time. Eventually, the joint pains subside, do not have the typical changes at the base of the organ involvement. Secondly, in most instances even in the face of new skin involvement but an fingernails that are common in the internal (systemic the condition is self-limited, and this definitely association with spine arthritis has been reported. sclerosis) disease: tiny blood vessel changes that applies to individual areas of morphea. In those Along the same lines, it is highly unlikely that require a special magnifying lens for proper cases, no treatment is required or advisable, except patients with localized scleroderma progress and detection. Another important question is whether for some emollients or, in some cases, mild topical develop severe systemic (internal) conditions such patients have what is called Raynaud’s phenomenon. steroids. Therefore, systemic treatment with a as rheumatoid arthritis, or lupus erythematosus, This refers to the inability of the patient’s hands to medication or other treatment interventions (for or systemic sclerosis (the internal form of withstand cold exposure, so that the fingers may be example, ultraviolet light), are reserved for more scleroderma). Still, it is important to recognize that come pale, or even experience numbness and pain, extensive forms of localized scleroderma. There morphea and other types of localized scleroderma and/or develop a temporary bluish appearance with are many publications that reference favorable may be present in several of these systemic even mild cold temperatures, or when touching results when using certain medications, but these conditions, including systemic lupus erythematosus, a cold beverage, or getting some item from the have truly not been proven to work. Examples of systemic sclerosis, and blistering diseases. An freezer. Patients with localized scleroderma these medications include: phenytoin (Dilantin), association has been found with a parasitic disease generally do not have Raynaud’s phenomenon, systemic corticosteroids (cortisone), antimalarial (toxoplasma). While this does not imply that patients which is present in the majority of patients with drugs (plaquenil, chloroquine), cyclosporine, with localized scleroderma evolve to develop these systemic sclerosis. d-penicillamine, antibiotics (presumably to treat an conditions, it does emphasize the need for follow-up underlying Lyme disease problem.) and vitamin E. and periodic evaluations, especially in patients who Several blood tests may be performed, which, when A previously used vitamin D-based therapy (with continue to have active skin problems or develop abnormal, help to determine how active the disease calcitriol) has now been proven not to work. There new issues. is and how extensive or prolonged it may become. is some recent good news for patients with extensive These blood tests include the number of blood disease who require proven medicines and other Are There Any Tests to eosinophils (cell normally present in our blood), the interventions. Methotrexate (as an anti-inflammatory Prove the Diagnosis of level of blood immune proteins (immunoglobulins), drug) has proved effective in children with active and various blood auto-antibodies: antinuclear and extensive (and deep) morphea. It has some side Localized Scleroderma? antibodies (ANA), antibodies to single-stranded effects and should only be used when truly necessary. The diagnosis of localized scleroderma is mainly DNA (ssDNA) and antihistones antibodies. These Ultraviolet light (there are different forms of this by visual recognition, though a biopsy often may tests are not specific for localized scleroderma and therapy, depending on the light wavelength being be done to show increased collagen deposition and may be abnormal in other conditions, including used) has also shown considerable promise in patients Funding for this brochure was provided by an unrestricted other suggestive changes upon microscopic analysis the internal type of scleroderma. However, with active disease. In other situations or when educational grant from Actelion Pharmaceuticals USA, Inc. of the skin. The biopsy may also help determine specific antibodies that are common in systemic these treatments fail, there are other medicines that whether the disease is still active. However, skin sclerosis (typically anti-centromere antibodies or can be tried in spite of the fact that they have not lesions may extend even when the biopsy does not anti-topoisomerase or SCL70) are expected to be been proven to work yet in careful clinical research show any significant changes. The process of taking negative in patients with localized scleroderma. studies. There is definite agreement that physical a skin biopsy involves first numbing the skin with therapy to preserve full motion of the affected joints an injection containing a numbing medicine (for What Medicines Are is important. example, lidocaine), and then taking a small (3-4 May 2015 mm or about 0.15 inches) circular area of skin with Available? Please note that this brochure is provided for a special “punch biopsy” device. Generally, unless There is no absolute cure for localized scleroderma. educational purposed only. It is not intended to one is dealing with a much deeper involvement of An absolute cure could be defined as not only substitute for informed medical advice. the skin, larger biopsies are not necessary. Stitches complete stabilization of the skin lesions, but also are optional when the biopsy site is very small. Any disappearance of the changes that have taken place in The Scleroderma Foundation wishes to thank Vincent Falanga, biopsy will result in a small scar. the skin, under the skin, and any associated cosmetic M.D., Boston University, for his contribution to this brochure. (arthralgia) during the course of their disease. The It’s important to look for physical signs of the or functional deformity. However, stabilization Our Three-Fold Mission Is pain is not limited to joints beneath the involved internal type of scleroderma (systemic sclerosis), (inactive disease) of localized scleroderma is indeed LOCALIZED skin, but may involve various joints, such as the such as thickening of the skin on all the fingers possible. The outlook and prognosis of localized Support, Education and knees, wrists and even the spine. (sclerodactyly), and thickening of the skin on the scleroderma is generally quite favorable. First, the Research SCLERODERMA Occasionally, this complication occurs before the face (without color changes). There are other clues disease is not fatal, does not appear to decrease appearance of lesions in the skin, causing some that can be identified during the initial examination. lifespan and, except for extremely rare situations, is confusion with rheumatoid arthritis for a short For example, patients with localized scleroderma not associated with another disease that has internal period of time. Eventually, the joint pains subside, do not have the typical changes at the base of the organ involvement. Secondly, in most instances even in the face of new skin involvement but an fingernails that are common in the internal (systemic the condition is self-limited, and this definitely association with spine arthritis has been reported. sclerosis) disease: tiny blood vessel changes that applies to individual areas of morphea. In those Along the same lines, it is highly unlikely that require a special magnifying lens for proper cases, no treatment is required or advisable, except patients with localized scleroderma progress and detection. Another important question is whether for some emollients or, in some cases, mild topical develop severe systemic (internal) conditions such patients have what is called Raynaud’s phenomenon. steroids. Therefore, systemic treatment with a as rheumatoid arthritis, or lupus erythematosus, This refers to the inability of the patient’s hands to medication or other treatment interventions (for or systemic sclerosis (the internal form of withstand cold exposure, so that the fingers may be example, ultraviolet light), are reserved for more scleroderma). Still, it is important to recognize that come pale, or even experience numbness and pain, extensive forms of localized scleroderma. There morphea and other types of localized scleroderma and/or develop a temporary bluish appearance with are many publications that reference favorable may be present in several of these systemic even mild cold temperatures, or when touching results when using certain medications, but these conditions, including systemic lupus erythematosus, a cold beverage, or getting some item from the have truly not been proven to work. Examples of systemic sclerosis, and blistering diseases. An freezer. Patients with localized scleroderma these medications include: phenytoin (Dilantin), association has been found with a parasitic disease generally do not have Raynaud’s phenomenon, systemic corticosteroids (cortisone), antimalarial (toxoplasma). While this does not imply that patients which is present in the majority of patients with drugs (plaquenil, chloroquine), cyclosporine, with localized scleroderma evolve to develop these systemic sclerosis. d-penicillamine, antibiotics (presumably to treat an conditions, it does emphasize the need for follow-up underlying Lyme disease problem.) and vitamin E. and periodic evaluations, especially in patients who Several blood tests may be performed, which, when A previously used vitamin D-based therapy (with continue to have active skin problems or develop abnormal, help to determine how active the disease calcitriol) has now been proven not to work. There new issues. is and how extensive or prolonged it may become. is some recent good news for patients with extensive These blood tests include the number of blood disease who require proven medicines and other Are There Any Tests to eosinophils (cell normally present in our blood), the interventions. Methotrexate (as an anti-inflammatory Prove the Diagnosis of level of blood immune proteins (immunoglobulins), drug) has proved effective in children with active and various blood auto-antibodies: antinuclear and extensive (and deep) morphea. It has some side Localized Scleroderma? antibodies (ANA), antibodies to single-stranded effects and should only be used when truly necessary. The diagnosis of localized scleroderma is mainly DNA (ssDNA) and antihistones antibodies. These Ultraviolet light (there are different forms of this by visual recognition, though a biopsy often may tests are not specific for localized scleroderma and therapy, depending on the light wavelength being be done to show increased collagen deposition and may be abnormal in other conditions, including used) has also shown considerable promise in patients Funding for this brochure was provided by an unrestricted other suggestive changes upon microscopic analysis the internal type of scleroderma. However, with active disease. In other situations or when educational grant from Actelion Pharmaceuticals USA, Inc. of the skin. The biopsy may also help determine specific antibodies that are common in systemic these treatments fail, there are other medicines that whether the disease is still active. However, skin sclerosis (typically anti-centromere antibodies or can be tried in spite of the fact that they have not lesions may extend even when the biopsy does not anti-topoisomerase or SCL70) are expected to be been proven to work yet in careful clinical research show any significant changes. The process of taking negative in patients with localized scleroderma. studies. There is definite agreement that physical a skin biopsy involves first numbing the skin with therapy to preserve full motion of the affected joints an injection containing a numbing medicine (for What Medicines Are is important. example, lidocaine), and then taking a small (3-4 May 2015 mm or about 0.15 inches) circular area of skin with Available? Please note that this brochure is provided for a special “punch biopsy” device. Generally, unless There is no absolute cure for localized scleroderma. educational purposed only. It is not intended to one is dealing with a much deeper involvement of An absolute cure could be defined as not only substitute for informed medical advice. the skin, larger biopsies are not necessary. Stitches complete stabilization of the skin lesions, but also are optional when the biopsy site is very small. Any disappearance of the changes that have taken place in The Scleroderma Foundation wishes to thank Vincent Falanga, biopsy will result in a small scar. the skin, under the skin, and any associated cosmetic M.D., Boston University, for his contribution to this brochure.

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