Skin-nontumor / Clinical Dermatology - Pemphigoid gestationis

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Skin-nontumor / Clinical Dermatology

Blistering disorders

Pemphigoid gestationis

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 9 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Rare, self-limiting, autoimmune, subepidermal bullous disease, occurring during or soon after pregnancy or in women taking oral contraceptives

Terminology
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● Formerly called herpes gestationis due to herpetiform nature of blisters, but disease is NOT related to herpes infection
● Called pruritis gravidarum when occurs without significant cutaneous stigmata

Epidemiology
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● Occurs in 1 per 50,000 pregnancies
● Rarely complicates hydatidiform mole and gestational choriocarcinoma
● Rarely present in postpartum period
● May follow a change in sexual partner

Sites
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● Pruritic lesions of abdomen, chest, back and extremities

Etiology
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● Due to circulating autoantibodies against placental collagen XVII (BP180, BPAG2) a hemidesmosomal transmembrane protein (J Cell Biochem 1999;72:356) and less frequently BP230

Clinical features
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● Usually urticarial papules, also blisters and rash
● Usually resolves within weeks to months after delivery
● Tends to recur with subsequent pregnancy
● Associated with premature delivery, small for gestational age infants

Prognostic factors
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● Poor prognostic factors: onset in first or second trimester and presence of blisters (Br J Dermatol 2009;160:1222)

Case reports
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● 29 year old woman with involvement of mother and newborn (Arch Gynecol Obstet 2009;279:235)

Treatment and prognosis
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● Oral and topical corticosteroids (J Am Acad Dermatol 2006;55:823)

Clinical images
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Abdominal lesions

36 year old woman at 24 weeks gestation

Exudative and erythematous lesions

Various images

Micro description
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● Similar to bullous pemphigoid - subepidermal blister, with eosinophils in lumen
● Marked edema in papillary dermis
● Perivascular infiltrate consists of lymphocytes, histiocytes and large numbers of eosinophils
● Eosinophilic spongiosis may be seen

Micro images
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H&E and C3

Perivascular infiltrate of lymphocytes and eosinophils, with eosinophils at dermoepidermal junction with associated early subepidermal vesiculation

Linear C3 staining

Virtual slides
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Herpes gestationis

Positive stains
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● Linear C3 deposits along cutaneous basement membrane; variable IgG deposition (Eur J Obstet Gynecol Reprod Biol 2009;145:138)
● ELISA may be more sensitive and specific than indirect immunofluorescence (Int J Dermatol 2008;47:1245)

Differential diagnosis
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● Pruritic urticarial papules of pregnancy: typically begins in stretch mark areas of abdomen and usually ends within 2 weeks after delivery; no antibody deposition
● Pregnancy prurigo: usually develops in the third trimester of pregnancy, presents with pruritic papules and nodules; histologic changes are those of low grade non specific spongiotic dermatitis

Additional references
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● eMedicine

End of Skin-nontumor / Clinical Dermatology > Blistering disorders > Pemphigoid gestationis

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