A Severe Case of Pediatric Linear IgA Bullous Dermatosis

Abstract

Linear IgA bullous dermatosis (LABD) is a rare immune-mediated subepidermalmblistering disease, most commonly affecting children between 4 and 5 years of age. Although generally self-limiting, some cases present diagnostic and therapeutic challenges.

  We report an 8-year-old boy who presented at age 5 with disseminated pruritic bullous lesions. Initial direct immunofluorescence was inconclusive, and diagnosis was confirmed only three years later upon repeat biopsy demonstrating linear IgA deposition at the basement membrane zone. The clinical course was complicated by recurrent secondary infections, drug-induced hepatitis from dapsone overdose, cyclosporine-induced disease aggravation, and treatment non-adherence. Disease control was ultimately achieved with dapsone and corticosteroids.

This case highlights the importance of repeat biopsy when clinical suspicion persists despite inconclusive findings, careful drug monitoring, and comprehensive caregiver education. A multidisciplinary approach is essential for managing severe pediatric LABD.