IgA Pemphigus

Excerpt

Immunoglobulin A (IgA) pemphigus, a rare autoimmune blistering disease, manifests as painful and pruritic vesiculopustular eruptions. These eruptions occur due to circulating IgA antibodies targeting keratinocyte cell surface components involved in cell-to-cell adherence. Although associated with various malignancies and chronic conditions, its precise etiology remains unclear. IgA pemphigus comprises 2 distinct subtypes: subcorneal pustular dermatosis and intraepidermal neutrophilic dermatosis. The subcorneal pustular dermatosis subtype presents with intercellular IgA deposition against desmocollin-1 glycoprotein, primarily in the upper epidermis, whereas the intraepidermal neutrophilic dermatosis subtype exhibits autoantibodies targeting desmoglein members in the cadherin superfamily, predominantly in the lower epidermis. Although IgA pemphigus typically has a milder disease course compared to IgG-driven pemphigus, it necessitates careful diagnosis and aggressive treatment with steroids and dapsone to prevent recurrence.