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Updated: Feb 20 2020

Herpes Gestationis / Pemphigoid Gestationis

  • 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
    • Associated conditions
      • thyroid disease, particularly Graves disease
      • premature birth
  • 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
  • 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
  • Prognosis
    • Good
    • May resolve spontaneously after delivery
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