Updated: 2/20/2020

Herpes Gestationis / Pemphigoid Gestationis

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Questions
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Evidence
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Topic
Snapshot
  • A 29-year-old G1P0 woman at 30 weeks pregnancy presents to her dermatologist for a rash that started on her abdomen. She reports having significant itch with this rash. She denies any new cosmetic or medication exposures in the past few months. Her pregnancy is otherwise healthy, without complications. On physical exam, she has multiple edematous pink papules and plaques on her abdomen without scale. There is one tense intact bulla noted on the left lower abdomen. A biopsy is done and sent for histology and direct immunofluorescence, which shows a subepidermal split.
Introduction
  • Overview
    • pemphigoid gestationis, also known as herpes gestationis, is an autoimmune bullous dermatosis that occurs during the second or third trimester of pregnancy, or in the immediate postpartum period
      • treatment is usually oral corticosteroids
  • Epidemiology
    • incidence
      • 1 in 50,000 pregnancies
    • demographics
      • pregnant women
    • risk factors
      • associated with HLA-DR3 and HLA-DR4
      • history of pemphigoid gestationis with previous pregnancies
  • Pathogenesis
    • mechanism
      • antibody to BPAG2, a component of the hemidesmosome
        • BPAG2 is also known as BP180
      • it is unclear why antibodies form
      • hypotheses include cross-reactivity between placental tissue and the skin
  • Associated conditions
    • thyroid disease, particularly Graves disease
    • premature birth
  • Prognosis
    • good
    • may resolve spontaneously after delivery
Presentation
  • Symptoms
    • periumbilical rash that progresses to tense bullae
    • significant pruritus
    • flares with delivery of neonate
  • Physical exam
    • inspection
      • edematous pink urticaria-like papules and plaques
      • tense vesicles or bulla
      • spares mucous membranes
Studies
  • Histology
    • dermal edema
    • subepidermal bullae
    • eosinophils
  • Direct immunofluorescence (DIF)
    • linear C3 (some with IgG) deposition at the basement membrane
Differential
  • Polymorphic eruption of pregnancy
    • key distinguishing factors
      • may also present with pruritic urticarial papules and plaques on the abdomen, but will not progress to bullae
      • DIF is negative
Treatment
  • Medical
    • oral corticosteroids
      • indications
        • symptomatic relief
        • typically, topical corticosteroids are not effective
Complications
  • Fetal risk
    • increased risk of premature birth

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Questions (1)

(M3.OB.15.28) A 29-year-old G2P1 woman at 37 weeks gestation presents to her primary care physician with a 3-day history of an abrupt onset of extremely pruritic, urticarial papules and blisters on the abdomen and trunk. Her pregnancy has otherwise been unremarkable and she is currently taking folate and a multivitamin. Her husband recently had an upper respiratory infection and her son recently had a fever and a rash. The patient has never been vaccinated nor has anyone else in the family. She has a history of cold sores and chickenpox. Her temperature is 98.0°F (36.7°C), blood pressure is 120/84 mmHg, pulse is 85/min, respirations are 14/min, and oxygen saturation is 98% on room air. Her physical exam is shown in Figure A. Which of the following is the most likely diagnosis? Tested Concept

QID: 103051
FIGURES:
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Bullous pemphigoid

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(0/6)

2

Herpes simplex virus

0%

(0/6)

3

Herpes zoster virus

0%

(0/6)

4

Pemphigoid gestationis

67%

(4/6)

5

Pemphigus vulgaris

33%

(2/6)

M 10 E

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Evidence (2)
EXPERT COMMENTS (1)
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