Dermatoses of pregnancy

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Chapter 60 Dermatoses of pregnancy

Misha D. Miller, MD, FACOG

Specific dermatoses of pregnancy

1. Name four pregnancy-specific dermatological disorders

• Pruritic urticarial papules and plaques of pregnancy

• Pemphigoid gestationis

• Atopic eruption of pregnancy

• Intrahepatic cholestasis of pregnancy

Ambros-Rudolph CM, Müllegger RR, Vaughan-Jones SA, et al: The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients, J AM Acad Dermatol 54(3):395–404, 2006.

2. What is pruritic urticarial papules and plaques of pregnancy?

Pruritic urticarial papules and plaques of pregnancy (also known as polymorphic eruption of pregnancy, or PUPPP) has an incidence of 1/200 pregnancies. The onset of PUPPP is usually in the third trimester. The pruritic, erythematous, papules and plaques are usually first seen in the abdominal striae (stretch marks) and then spread to the chest, trunk, and extremities. The papules and plaques typically spare the palms, soles, face, and mucous membranes. Large vesicles or bullae are uncommon, though pinpoint vesicles may be seen (Fig. 60-1).

Figure 60-1. Pruritic urticarial papules and plaques of pregnancy (PUPPP). A, Erythematous papules in the striae of a 21-year-old primigravida woman. B, Urticarial papules and plaques that are not associated with striae on the thighs of a woman with PUPPP.

Ahmadi S, Powell FC: Pruritic urticarial papules and plaques of pregnancy: current status, Austr J Dermatol 46:53–60, 2005.

Matz H, Orion E, Wolf R: Pruritic urticarial papules and plaques of pregnancy: polymorphic eruption of pregnancy (PUPPP), Clin Dermatol 24(2):105–108, 2006.

3. Does PUPPP have any associated morbidity?

The pruritis associated with PUPPP can be very uncomfortable for the mother. There are no fetal sequelae. The skin lesions and pruritus associated with PUPPP usually spontaneously resolve within 1 or 2 weeks after delivery.

4. How is PUPPP treated?

Topical corticosteroids and oral antihistamines are the most common methods of treatment. However, the efficacy of oral antihistamines in treating the pruritus of PUPPP is questionable. The use of oral corticosteroids is rare.

Matz H, Orion E, Wolf R: Pruritic urticarial papules and plaques of pregnancy: polymorphic eruption of pregnancy (PUPPP), Clin Dermatol 24(2):105–108, 2006.

5. From which dermatosis of pregnancy must PUPPP be differentiated?

Pemphigoid gestationis. It may be difficult to clinically differentiate PUPPP from pemphigoid gestationis.

6. What is pemphigoid gestationis?

Pemphigoid gestationis (also called herpes gestationis or gestational pemphigoid) is an autoimmune subepidermal bullous disorder of pregnancy that may start in the 2nd trimester, the 3rd trimester, or in the postpartum period. The skin lesions are characterized by urticarial papules and plaques, which then progress into painful bullae. The lesions of pemphigoid gestationis initially present periumbilically (Fig. 60-2A), and then spread to involve the trunk and extremities (Fig. 60-2B), usually sparing the face.

Figure 60-2. Pemphigoid gestationis. A, Umbilical urticarial plaques and tense blisters in a woman with pemphigoid gestationis. B, Intertriginous area between fingers demonstrating tense blisters. This is another commonly affected site in pemphigoid gestationis.

(Courtesy of the Fitzsimons Army Medical Center teaching files.)

Semkova K, Black M: Pemphigoid gestationis: current insights into pathogenesis and treatment, Eur J Obstete Gynecol Reprod Biol 145(2):138–144, 2009.

7. What are the antigens associated with the development on pemphigoid gestationis?

Bullous pemphigoid antigen 2 (BPAG2) is a 180 kDal transcellular glycoprotein that is part of the hemidesmosome (a structure that binds epithelial cells to the basement membrane of the epidermis). IgG binds to BPAG2 and triggers the classic complement pathway leading to a deposition of C3 along the basement membrane zone (Fig. 60-3). Deposition of complement along the basement membrane zone leads to a recruitment of inflammatory cells, particularly eosinophils. This cascade ultimately leads to a release of proteolytic enzymes that cleave portions of the epidermis from the dermis.

Figure 60-3. Pemphigoid gestationis. Direct immunofluorescent study demonstrating linear C3 along the basement membrane zone. Less commonly, linear IgG is also seen.

Key Points: Pemphigoid Gestationis

1. Pemphigoid gestationis is an autoimmune disease that occurs during pregnancy, and it is characterized by IgG₁ antibodies directed at bullous pemphigoid antigen–1 in the basement membrane zone.

2. Direct immunofluorescent study demonstrates linear C3 and/or IgG along the basement membrane zone and is the diagnostic test of choice to establish the diagnosis.

3. The primary lesions of pemphigoid gestationis consist of urticarial plaques and/or blisters.

4. The umbilical area is preferentially affected in pemphigoid gestationis and can be a clue to the diagnosis.

5. Neonates of mothers with pemphigoid gestationis may demonstrate a lower birth weight and may occasionally demonstrate transient blisters, but are otherwise healthy.

8. Which histocompatibility leukocyte antigen (HLA) types have been associated with pemphigoid gestationis?

HLA-DR3 and HLA-DR4.

Powell AM, Sakuam-OyamaY, Oyama N, et al: Usefulness of BP180 NC16a enzyme-linked immunosorbent assay in the serodiagnosis of pemphigoid gestationis and in differentiating between pemphigoid gestationis and pruritic urticarial papules and plaques of pregnancy, Arch Dermatol 141(6):705–710, 2005.

9. Compare PUPPP and pemphigoid gestationis.

See Table 60-1.

Table 60-1. Compare and Contrast PUPPP and Pemphigoid Gestationis

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	PUPPP	PEMPHIGOID GESTATIONIS
Clinical presentation	Pruritic erythematous papules and plaques Initial lesions present in the abdominal striae, spreading to the trunk and extremities; vesicles may be present Usually spares the palms and soles	Urticarial papules, plaques, and blisters Initial lesions start periumbilically and spread to the trunk and extremities The palms and soles are commonly involved
Direct immunofluorescence

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