Linear IgA Bullous Dermatosis: A Rare Clinicopathologic Entity with an Unusual Presentation.

Abstract

Linear immunoglobulin A bullous dermatosis is a rare autoimmune mucocutaneous disorder caused by immunoglobulin A autoantibodies produced against several different antigens in the basement membrane zone. Clinically, it is characterized by tense vesicles or bullae, which on histopathological exam demonstrate subepidermal blister with a predominantly neutrophilic infiltrate. A smooth, linear pattern of immunoglobulin A deposition in the basement membrane zone on direct immunofluorescence is considered the gold standard for establishing a diagnosis. Treatment consists of dapsone or sulfapyridine. The authors report a 60-year-old woman who presented with pruritic erythematous patches and plaques on her trunk, back, and legs without blisters, who was diagnosed with eczema for several months with no response to prior treatments. A biopsy was performed, which was consistent with linear immunoglobulin A bullous dermatosis and later confirmed by direct immunofluorescence studies. The authors present this case to increase awareness of this rare disease, which could manifest in a nonclassical, nonblistering fashion.

• Authors: Soham Chaudhari, Narciss Mobini
• Journal: The Journal of clinical and aesthetic dermatology (J Clin Aesthet Dermatol) Vol. 8 Issue 10 Pg. 43-6 (Oct 2015) ISSN: 1941-2789 [Print] United States
• PMID: 26557220 (Publication Type: Journal Article)