Uncovering the mask: Linear IgA bullous dermatosis treated with dapsone in a Filipino adult male

Authors: Jennifer Lavina T. Ngo, MD, MBA; Martha Joy B. Tapales, RPh, MD, DPDS, Mae R. Quizon, MD, FPDS, Alma Gay Concepcion T. Amado, MD, FPDS

Abstract

Introduction: Linear IgA bullous dermatosis is a chronic, acquired autoimmune blistering disease characterized by nonspecific lesions that may be found in other diseases belonging to the same category.

Case summary: This case presents a 41-year old male with multiple, well-defined, clear, fluid-filled, tense vesicles with crusted erosions and urticarial plaques on the face, neck and later involved the chest, back, anogenital area, and extensors in an annular pattern. Dermatopathology revealed a subepidermal split with neutrophil predominance supported by a direct immunofluorescence showing linear IgA deposition at the dermoepidermal junction. The patient was initially given oral corticosteroids. After adequate G6PD levels were ascertained, dapsone was subsequently started while tapering oral corticosteroid, with resolution of lesions.

Conclusion: Linear IgA bullous dermatosis is an autoimmune blistering disease that presents with tense bullae and crusted erosions on the face, trunk, genitals, and extensors with a peak incidence in children and adults. The disease may mimic presentations of other autoimmune blistering diseases, and histopathology may not always yield specific results, making DIF the gold standard for diagnosing LABD. This case highlights the clinical heterogeneity of LABD and the failure to reach full clinical control with use of second-line agents. A complete history, physical exam, and diagnostic work-up that include both histopathologic findings and direct immunofluorescence are needed to accurately diagnose and unmask the disease

 

Citation

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Keywords

Linear IgA bullous dermatosis, autoimmune blistering disease, dapsone

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