II. Epidemiology

  1. Rare
  2. Age of Onset: Under age 5 years

III. Pathophysiology

  1. Autoimmune bullous disorder
  2. Variant of Linear IgA Dermatosis (seen in adults)

IV. Symptoms

  1. Pruritus and burning Sensation

V. Signs

  1. Sudden onset of tense inflamed Blisters over red base
    1. May appear as collarettes of Blisters
  2. Distribution
    1. Most commonly found on genitalia
    2. Also may involves face and perioral skin
    3. Oral Mucosa involved in 50% of cases

VI. Labs

VII. Management

VIII. Course

  1. Resolves within 2 years of onset in most cases

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