Common Dermatoses of Pregnancy

Home , Dermatoses of pregnancy, Impetigo herpetiformis, Postpartum period, Prurigo gestationis

Ramsay S. Farah, M.D. Associate Professor of Medicine and Pathology Farah Dermatology and Cosmetics, LLC Upstate Medical University Common Dermatoses of Pregnancy

• Pregnancy is a time of significant and complex physiologic changes • Some of these changes are due to the de novo production of a variety of protein and steroid hormones by the fetoplacental unit as well as by increased activity of the maternal pituitary, thyroid, and adrenal glands

Common Dermatoses of Pregnancy

• The chemistry, function, and metabolism of these hormones is quite complex. • The levels of these hormones are of diagnostic significance in certain obstetric conditions and complications, but their exact impact on cutaneous physiology as well as their influence on the immunology of the skin remains essentially unknown. Common Dermatoses of Pregnancy

Well defined eruptions: -PUPP (pruritic urticarial papules and plaques of pregnancy) -Pemphigoid (herpes) gestationis -Impetigo Herpetiformis -Recurrent cholestasis of Pregnancy

Poorly defined eruptions: -Prurigo gestationis -Papular dermatitis of pregnancy -Follicular eruption of pregnancy -Autoimmune progesterone dermatitis Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• Otherwise known as polymorphic eruption of pregnancy • The most common gestational dermatoses with an incidence of 1 in 160 pregnancies • Seen most frequently in the late trimester or occasionally in the early postpartum period Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• The mean duration is 6 weeks, but the rash is not severe for more than one week.

• Unlike urticaria, the eruption remains fixed and increases in intensity, clearing in most cases before or within one week of delivery

• Recurrences with future pregnancies is unusual Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP) • There are no fetal or maternal complications • Infants do not develop the eruption • Etiology is unknown. It has been associated with increased maternal-fetal weight gain and twin pregnancy, and so it was postulated to be related to abdominal distention. Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• The eruption begins suddenly, developing first on the abdomen in 90% of patients • The initial lesions maybe confined to the striae Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• The initial lesions begin as red papules that are often surrounded by a narrow, pale halo Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• Early in the course, the lesions often predominate in the periumbilical area with sparing of the umbilicus Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• The lesions then increase in number and may become confluent, resulting in broad areas of erythema that spread to involve the buttocks and thighs. • The papules maybe discrete at this point. Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• In other instance the lesions may develop into erythema multiforme like lesions or lesions that look like herpes gestationis Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• Histologic features include a superficial perivascular infiltrate of lymphocytes, monocytes and eosinophils • Variable dermal edema • Variable epidermal spongiosis Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• Direct immunofluorescence of lesional or peri- lesional skin is negative • This can be used as a distinction with herpes gestationis Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP)

• Direct immunofluorescence of lesional or peri- lesional skin is negative • This can be used as a distinction with herpes gestationis Pruritic Urticarial Papules and Plaques of Pregancy (PUPP) • Treatment: The goal of treatment is to relieve pruritus, halt progression of the eruption, and hasten its resolution. • Most patients respond to frequent (5-6 times per day) application of potent topical steroids. • Once the eruptions is controlled, one can taper to less potent topical steroids Pruritic Urticarial Papules and Plaques of Pregancy (PUPP)

• Patients with extensive eruptions or those refractory to treatment respond to a tapering course of systemic steroids (starting at 40mg/day and tapering by 5 mg every 2-3 days) • Antihistamines may also be used for the control of pruritus Pruritic Urticarial Papules and Plaques of Pregancy (PUPP)

• Differential Diagnosis: • Erythema Multiforme • Drug eruptions • Contact dermatitis • Insect bites • Scabies • Urticaria • Herpes Gestationis Pruritic Urticarial Papules and Plaques of Pregancy (PUPP) Pruritic Urticarial Papules and Plaques of Pregancy (PUPP) Pruritic Urticarial Papules and Plaques of Pregancy (PUPP) Pruritic Urticarial Papules and Plaques of Pregancy (PUPP)

Pruritic Urticarial Papules and Plaques of Pregancy (PUPP)

Pruritic Urticarial Papules and Plaques of Pregancy (PUPP) Herpes Gestationis (Pemphigoid Gestationis) • An extremely pruritic polymorphic inflammatory, subepidermal bullous eruption of pregnancy and the postpartum period • Occurs in fewer than 1 in 50,000 pregnancies • Definitive diagnosis can be made with specific immunopathologic studies.

Herpes Gestationis (Pemphigoid Gestationis)

• Usually begins from the fourth to seventh month of pregnancy, but the onset has been reported during the first trimester and in the immediate postpartum period • Postpartum exacerbations as well as flares with the first few postpartum menstrual periods are common • May or may not recur in subsequent pregnancies; if it does, it is likely to begin earlier Herpes Gestationis (Pemphigoid Gestationis)

• Many authors are calling this disease pemphigoid gestationis because it has 1) Immunologic similarities to bullous pemphigoid and 2) No relationship with herpes virus infections. Herpes Gestationis (Pemphigoid Gestationis)

• Lesions vary from erythematous, edematous papules to large, tense bullae with many intermediate forms including small vesicles, confluent papules and vesicles and urticarial like plaques; with and without grouping, bullae, erosions and crusts Herpes Gestationis (Pemphigoid Gestationis)

• Involves the abdomen usually, but may involve other areas as well (including palms, soles, chest, back and face) • Begins as edematous plaques occurring in crops on the abdomen and extremities • Mucous membrane involvement is rare Herpes Gestationis (Pemphigoid Gestationis)

• These then coalesce into bizarre polycyclic rings covering wide areas of the skin Herpes Gestationis (Pemphigoid Gestationis)

• Widespread urticarial plaques characterized by round shape and by polycyclic outlines. Herpes Gestationis (Pemphigoid Gestationis)

• These then coalesce into bizarre polycyclic rings covering wide areas of the skin Herpes Gestationis (Pemphigoid Gestationis)

• As with bullous pemphigod, within days to weeks the tense blisters evolve from the urticarial like plaques, rupture and leave slowly healing, denuded areas that heal without scarring Herpes Gestationis (Pemphigoid Gestationis)

• It may leave postinflammatory hyperpigmentation. • Spontaneous clearing may be seen in the latter parts of the pregnancy, but flares are seen at the time of delivery in 75- 80% of cases • Mild recurrences may occur with menses or with the use of oral contraceptives Herpes Gestationis (Pemphigoid Gestationis) • Etiology: It is postulated that IgG antibodies are initiated in response to an antigenic stimulus peculiar to pregnancy

• These antibodies have a specificity for a 180 kDa antigen (BPAG2 or type XVII collagen) that is a hemidesmosomal protein in the basement membrane Herpes Gestationis (Pemphigoid Gestationis)

• Once deposited in the BM zone, these antibodies activate the complement cascade

• The IgG antibody can in most cases, cross the placenta, hence the occasional genesis of transient blistering in the infant Herpes Gestationis (Pemphigoid Gestationis) Herpes Gestationis (Pemphigoid Gestationis) Herpes Gestationis (Pemphigoid Gestationis)

• Treatment: is designed to suppress blister formation and to relieve the intense pruritus. • Achieved by giving 20-40mg of prednisone in divided doses daily. • Exacerbation of the pruritus and blistering commonly occur at parturition and may then require and increase in predinisone – this is then gradually tapered in the postpartum period. Herpes Gestationis (Pemphigoid Gestationis)

• Exacerbations at the time of menses may require a temporary increase in dosage • A few patients do not require systemic prednisone and can be managed with antihistamines and topical steroids or emollients • At other extremes, some individuals after parturition require azathioprine in addition to prednisone to control their disease Herpes Gestationis (Pemphigoid Gestationis)

• Alternatives to steroids or adjuvants (dapsone, MTX,Gold, Cyclophosphamide, plasmapheresis) are anecdotal and the experience is variable • No compelling argument for inducing delivery has been made. • The cutaneous lesions noted in infants are of a transient nature and require no therapy. Herpes Gestationis (Pemphigoid Gestationis)

• Differential Diagnosis: - PUPP - Erythema Multiforme -Dermatitis Herpetiformis - Allergic contact dermatitis - Drug hypersensitivity - Bullous Pemphigoid Herpes Gestationis (Pemphigoid Gestationis) Herpes Gestationis (Pemphigoid Gestationis) Herpes Gestationis (Pemphigoid Gestationis) Herpes Gestationis (Pemphigoid Gestationis) Herpes Gestationis (Pemphigoid Gestationis) Herpes Gestationis (Pemphigoid Gestationis)

• What are the differences with Bullous Pemphigoid? 1) The patient population at risk 2) The provocative factors (estrogens or progesterone) 3) Almost universal tendency for remission following pregnancy and between pregnancies 4) Ultrastructural studies suggest that blister formation in the basement membrane zone occurs in different areas in both conditions and the basement membrane zone antigenic molecules are actually different (230- 240 kd component of the hemisdesmosome for B.P. and a 180kd transmembrane protein for HG) Herpes Gestationis (Pemphigoid Gestationis)

Comparision with PUPP: 1) +Direct Immunofluoresence studies 2) Recurs with subsequent pregnancies 3) Infants may have a similar transient dermatitis 4) No particular sparing of the umbilicus

Impetigo Herpetiformis

Impetigo herpetiformis is form of pustular psoriasis that occurs during pregnancy and may be life threatening

It is exceedingly rare and approximately only 100 cases have been reported.

The disorder was first described by von Hebra in 1872 in five pregnant women, four of whom died Impetigo Herpetiformis

Tends to occur in the third trimester, although some cases have been reported as early as the first trimester Many women have no family or personal history of psoriasis Re-occurence in subsequent pregnancies have been reported Impetigo Herpetiformis

• The earliest lesions are erythematous patches occurring in the groin, axillae, and anterior and posterior neck. • At their margins these patches are studded with tiny superficial pustules Impetigo Herpetiformis

• Most patients have constitutional symptoms, the most common being fever and chills, often accompanied by nausea, vomiting, and diarrhea Impetigo Herpetiformis Impetigo Herpetiformis

• The lesions expand by peripheral extension with new pustules at the interior of the expanding lesions breakdown, resulting in crusting or impetiginization. • Pruritis is unusual Impetigo Herpetiformis

• In some cases mucous membranes may be affected and subungal pustules can cause onycholysis Impetigo Herpetiformis Impetigo Herpetiformis Impetigo Herpetiformis

Labs: Elevated white blood cell counts and sedimentation rates are quite common

When these occur in the presence of fever, infection must be ruled out.

The unopened pustules are sterile, but the skin may become secondarily infected.

Decreased serum calcium and serum albumin levels are also sometimes found. Impetigo Herpetiformis

The disease tends to remit promptly after delivery but may recur in subsequent pregnancies

There maybe an increased risk of fetal morbidity and mortality associated with placental insufficiency Impetigo Herpetiformis

Treatment: Systemic steroids are the treatment of choice with doses up to 60 mg/day being necessary to control the eruption Once under control, the prednisone should be tapered judiciously, since there is a risk of sudden exacerbation if the steroids are withdrawn too quickly. Impetigo Herpetiformis

Treatment: Patients should be monitored for systemic and cutaneous infections and treated with appropriate antibiotics Serum calcium and albumin levels should also be followed and replacement therapy undertaken if levels become too low. Prurigo Gravidarum

• Otherwise known as recurrent cholestasis of pregnancy or benign recurrent intrahepatic cholestasis • This is a hepatic condition that usually occurs late in pregnancy and demonstrates cutaneous manifestations of pruritus (and in some cases) jaundice • Otherwise there is no primary cutaneous lesions, although secondary excoriations occur Prurigo Gravidarum

• The initial cutaneous manifestation is pruritus • This maybe either localized or generalized • If jaundice is to occur, pruritus preceeds it by 2-4 weeks • In severe cases, excoriations, jaundice, nausea, vomiting, and right upper quadrant abdominal discomfort may be observed • Tends to remit soon after delivery, but typically recurs in subsequent pregnancies • It may occur in some, after exposure to oral contraceptives. Prurigo Gravidarum

• Some reports have suggested that there is an increase in prematurity, stillbirth, and postpartum hemorrhage. • The incidence of these adverse events seems highest in patients with both pruritus and jaundice Prurigo Gravidarum

• Pathogenesis: The exact cause is unknown, but it is believed to be hormonally induced (it is not clear whether estrogens or progesterone is the primary inciting agent, but they may work synergistically It’s elicitation by OCP supports this hypothesis The precise pathophysiology of the cholestasis is unknown. Prurigo Gravidarum

• Treatment: Attempts should be made to control pruritus with bland emollients and topical antipruritic agents. In many instances these provide adequate relief Antihistamines are affective. Cholestyramine may occasionally be effective Some advocate the administration of vitamin K before delivery to diminish the risk of postpartum hemorrhage Prurigo Gravidarum Other eruptions associated with pregnancy

• A number of other eruptions have been reported to be specifically associated with pregnancy. However, many of these eruptions have been incompletely characterized clinically, histologically, and immunopathologically. • In addition, most reports of these eruptions have involved relatively small numbers of patients and in some cases have lacked confirmation. Postpartum Telogen Effluvium

• Normal hairs can be classified according to cyclical phases of growth.

• Anagen hairs are growing hairs • Catagen hairs are transitioning from the growing phase to the resting phase • Telogen hairs are resting hairs, which remain in the follicle for variable lengths of time before falling out. Postpartum Telogen Effluvium

• A number of events have been documented that prematurely terminate anagen and cause an abnormally high number of hairs to enter the telogen or resting phase. • The hair follicle is not diseased, but has its biologic clock reset and undergoes a normal involutional process. • Resting hairs on the scalp are retained for about 100 days before they are shed Postpartum Telogen Effluvium Postpartum Telogen Effluvium

• Postpartum telogen effluvium has been found to begin between 2-6 months postpartum. • Often hair loss is noted to begin over anterior third of the scalp, although loss is in a diffuse pattern Postpartum Telogen Effluvium

• The hair loss may continue for some 2-6 months or longer • Patients note “lots of hairs coming out by the roots”

Postpartum Telogen Effluvium

• Treatment: Reassurance and time. • Minoxidil (Rogaine)may affect telogen effluvium by initiating anagen in many telogen follicles. This causes immediate telogen release and a brief, short onset telogen effluvium. Melasma (Chloasma Faciei)

• Melasma (the “mask of pregnancy) is characterized by brown patches, typically on the malar prominences,forehead, upper lips and chin • The pigment develops slowly without signs of inflammation and maybe faint or dark Melasma (Chloasma Faciei)

• There are three clinical patterns: 1) Centrofacial 2) Malar 3) Mandibular

Melasma (Chloasma Faciei)

• There are four types based on Wood’s light examination: 1)An epidermal type that shows enhancement of color contrast with Wood’s lamp. 2)A dermal type that does not 3)A mixed type that shows no or slight enhancement 4)Woods lamp inapparent, which is seen in dark individuals The epidermal type responds to depigmentation agents; the dermal types resist the action of bleaching agents. Melasma (Chloasma Faciei)

• The pigmented patches are usually quite well demarcated. • Increased pigment may also occur at the same time on the nipples and about the external genitalia • It tends to affect darker skinned individuals • Rarely, it maybe found on the forearms. Melasma (Chloasma Faciei)

• Melasma occurs frequently during pregnancy and at menopause (during second or third trimester) • Gradually fades after pregnancy, but recurs and darkens with subsequent pregnancies • It maybe related to ovarian disorders and other endocrine disorders. • There is a strong association with birth control pill use (use of dilantin may also induce melasma) Melasma (Chloasma Faciei) Melasma (Chloasma Faciei)

Treatments: • Sunlight appears to be an exaccerbating factor, therefore sunavoidance is critical in its treatment. • Bleaching creams (4% hydroquinones) and retinoids • The combination of hydroquinone, retinoids, and mild steroid (Kligman’s formula) is excellent • Jessner’s solution, glycolic acid peels, azeleic acid, kojic acid, are additional considerations. • Laser treatment has limited role because of the risk of postinflammatory hyperpigmentation. Melasma (Chloasma Faciei)

• Normally, up to 2 months are required to begin a response to treatment, and up to 6 months to complete the process. • Once epidermal melasma has cleared and is no longer apparent by Wood’s lamp, then hydroquinone and retinoids should be discontinued, but a broad spectrum, opaque sunscreen should be used. • Avoidance of medications that cause melasma is of course, part of the treatment. Telangiectasia

• Telangiectasia are permanently dilated, small blood vessels consisting of either venules, capillaries, or arterioles • They appear as single strands, in groups as small macules, or with a central punctum (spider angioma) • They are usually of a cosmetic problem only and rarely bleed Telangiectasia Telangiectasia

• Spider angiomas occur most commonly on the exposed surface of the face and arms • They increase in number during pregnancy and with liver disease. It is thought that higher than normal estrogen levels stimulate their appearance. Telangiectasia

• Treatment: Electordessication

Lasers (Pulse dye laser) Striae Distensae (Striae Gravidarum)

• Striae are depressed lines or bands of thin, reddened skin, which later become white, smooth, shiny, and depressed. • They occur on the abdomen during and after pregnancy Striae distensae (Striae Gravidarum) Striae Distensae (Striae Gravidarum)

• Treatments: Topical retinoids

Vascular lasers (pulse dye laser) and smooth beam laser (diode laser) Pyogenic Granuloma (Ganuloma Gravidarum)

A pyogenic Granuloma is a small, solitary, sessile or pedunculated, rasberry like vegetation of exuberant granulation tissue

Pyogenic Granuloma (Ganuloma Gravidarum)

The lesion is a dull red color and occurs on the exposed surfaces of the hands, forearms, face, or at sites of trauma. Pyogenic Granuloma (Ganuloma Gravidarum)

The lesion also occurs in the mouth, especially on the gingiva, most often in pregnant women (granuloma gravidarum)

Pyogenic Granuloma (Ganuloma Gravidarum)

Etiology is unknown: The occurrence with pregnancy or with birth control pills, and spontaneous regression after pregnancy all suggest a hormonal etiology Pyogenic Granuloma (Ganuloma Gravidarum)

Histologically the epidermis is thinned. The growth is composed of numerous newly formed capillaries arising from a central “feeder”. The capillaries are lined by a single layer of endothelial cells There is also an edematous, fibroblastic proliferation in the stroma Pyogenic Granuloma (Ganuloma Gravidarum)

Pyogenic granuloma is usually easily diagnosed. The ddx includes melanoma, atypical fibroxanthoma, metastatic carcinoma, or Kaposi’s sarcoma Pyogenic Granuloma (Ganuloma Gravidarum)

Treatment is surgical excision. Usually shave excision and electrodessication of the base.

Common Dermatoses of Pregnancy

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