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Skin - nontumor

Blistering disorders - Pemphigoid gestationis

 

Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.

Revised: 31 October 2009

Last major update: October 2009

Copyright: (c) 2002-2009, 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

 

Epidemiology

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● Occurs in 1 per 50,000 pregnancies

 

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, a hemidesmosomal transmembrane protein (J Cell Biochem 1999;72:356)

 

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

ELISA serum testing may be more sensitive and specific than indirect immunofluorescence (Int J Dermatol 2008;47:1245)

 

Prognostic factors

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● Poor prognostic factors: onset in the 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 transfer to infant (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                                             Exudative and erythematous lesions

 

 

      

Various images

 

Microscopic description  (Histopathology)

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● Similar to bullous pemphigoid - subepidermal blister, with eosinophils in lumen

● Marked edema in papillary dermis

 

Micro images

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H&E and C3                          Bullous pemphigoid                                     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)

 

Differential diagnosis

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● Pruritic urticarial papules of pregnancy: typically begins in stretch mark areas of the abdomen and usually ends within 2 weeks after delivery; no antibody deposition

 

Additional references

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eMedicine

 

End of Skin - nontumor > Blistering disorders > Pemphigoid gestationis

 

 

 

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