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Updated: Oct 23 2016

Dermatitis Herpetiformis

  • Snapshot
    • A 33-year-old man has a 2 year history of recurrent blistering, and itchy eruptions, especially on his forearms. There are no exacerbating factors. He reports no gastrointestinal symptoms. He has a past medical history of hypothyroidism.
  • Introduction
    • Chronic autoimmune skin disease
      • IgA anti-transglutaminase immune complex
      • considered cutaneous manifestation of celiac disease
    • Epidemiology
      • males affected more than females (2:1)
      • onset in adulthood
    • Associated conditions
      • other autoimmune conditions such as thyroid disease
  • Presentation
    • Skin findings
      • severely itchy and burning blistering eruption
      • clustered papules, vesicles, or excoriations
      • symmetric distribution, especially on elbows and knees
      • oral lesions uncommon
    • Other findings
      • typically no gastrointestinal symptoms
  • Evaluation
    • Skin biopsy
      • gold standard
      • direct immunofluorescence showing deposits of IgA at tips of dermal papillae
      • subepidermal vesicles with neutrophilic infiltrate
    • Small bowel biopsy
      • villous atrophy
    • Serologic testing
      • endomysial antibodies
      • tissue transglutaminase antibodies
      • epidermal transglutaminase antibodies
  • Differential Diagnosis
    • Scabies (intense unremitting itching)
    • Atopic dermatitis
  • Treatment
    • Lifestyle modifications
      • gluten-free diet
    • Pharmacological
      • dapsone
  • Prognosis, Prevention, and Complications
    • Prognosis
      • chronic disease
      • spontaneous remission in 1/3 of cases
    • Prevention
      • can be well-controlled with dapsone or gluten-free diet
    • Complications
      • typically none
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