DOI: https://doi.org/10.36347/sjmcr.2026.v14i08.012

Pages: 1815-1819

Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease characterized by linear IgA deposition along the basement membrane zone. While dapsone remains the cornerstone of treatment, a subset of adult patients develops severe, treatment-refractory disease requiring prolonged systemic corticosteroid therapy, and the role of rituximab in this setting remains poorly documented. We report the case of a 34-year-old woman with severe mucocutaneous LABD who failed to achieve disease control despite 18 months of treatment with dapsone, high-dose systemic corticosteroids, and doxycycline, resulting in persistent corticosteroid dependence and significant impairment in quality of life. Following confirmation of the diagnosis by repeat histopathology and direct immunofluorescence, rituximab was initiated using the pemphigus protocol (two 1-g infusions administered two weeks apart), followed by maintenance infusions every six months. A marked and sustained clinical improvement became evident from the second treatment cycle, allowing complete withdrawal of systemic corticosteroids and doxycycline, and at the latest follow-up the patient remained clinically stable on dapsone monotherapy, with only minimal residual cutaneous activity. This case highlights rituximab as a promising steroid-sparing rescue therapy for severe refractory adult LABD and contributes to the limited evidence supporting its use in this uncommon autoimmune bullous disease; further studies are needed to establish its optimal treatment regimen and long-term efficacy.