(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. 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