Linear immunoglobulin A/immunoglobulin G bullous dermatosis with celiac disease in a 26-year-old Filipino male

Abstract

Linear immunoglobulin A/immunoglobulin G bullous dermatosis (LAGBD) is a rare form of autoimmune blistering disease characterized by circulating anti-keratinocyte cell surface antibodies from both the immunoglobulin A and immunoglobulin G. It presents as blisters, erosions, and pustules with erythematous lesions, primarily on the trunk and extremities. A 26-year-old Filipino male presented with a 4-year history of multiple pruritic vesicles on an erythematous base on the mentum spreading on the face, scalp, neck, upper trunk, and upper extremities. Skin biopsy and direct immunofluorescence suggest a diagnosis of LAGBD. The patient underwent esophagogastroduodenoscopy, which revealed duodenal villous atrophy. A small bowel biopsy revealed duodenal villous atrophy with a predominance of lymphocytes. He was treated with dapsone for two years with remarkable improvement. He was also advised to do a strict, lifelong gluten-free diet. After a 5-year follow-up, he only experienced occasional very mild flares treated with topical clobetasol 5% cream. We present a rare case of a patient with LAGBD with celiac disease. Systemic involvement, particularly gastroenteropathy, should also be investigated. Dapsone and a gluten-free diet have been the primary treatment modalities for this case.