Linear IgA dermatosis

Full Definition

Linear IgA dermatosis is an autoimmune blistering disease predominantly affecting young children, characterized by linear deposition of IgA antibodies at the basement membrane zone. It presents with tense bullae and urticarial plaques, often in a distinctive 'cluster of jewels' or 'string of pearls' pattern around a central blister. The condition commonly involves the perioral and anogenital areas and typically responds well to dapsone therapy.

In Context

• "The 'string of pearls' distribution of bullae around the child's mouth was pathognomonic for linear IgA dermatosis." — Dermatopathology report
• "Linear IgA dermatosis in children typically has an excellent prognosis with most cases resolving by adolescence." — Patient counseling documentation