A 55-year-old woman presents with recurrent annular clusters of pustules over the trunk and flexures without mucosal involvement. Histology shows a subcorneal pustule filled with neutrophils. DIF reveals intercellular IgA throughout the epidermis. She improves markedly on dapsone. What is the diagnosis?
- A Subcorneal pustular dermatosis type of IgA pemphigus ✓
- B Intraepidermal neutrophilic type of IgA pemphigus
- C Pemphigus foliaceus
- D Linear IgA bullous dermatosis
Explanation
IgA pemphigus has two main patterns. The subcorneal pustular dermatosis type shows a subcorneal neutrophilic pustule with intercellular IgA targeting desmocollins, and responds well to dapsone. The intraepidermal neutrophilic type shows pustules at various epidermal levels. Linear IgA bullous dermatosis produces a subepidermal blister with linear basement membrane zone IgA, not an intercellular pattern, which excludes option D.
Reference: Bolognia, Schaffer and Cerroni, Dermatology, 5th ed.
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