Abstract

INTRODUCTION: Linear IgA bullous dermatosis (LABD) is a rare extraintestinal manifestation of inflammatory bowel disease (IBD). This autoimmune subepidermal disease presents with bullae, erosions and vesicles. LABD is characterized by the immunopathologic finding of linear IgA deposits in the basement membrane along the dermo epidermal junction. There is an uncommon association of LABD with IBD and PSC. CASE DESCRIPTION/METHODS: A 36-year-old male, with a history of Ulcerative Colitis (UC) presented for initial evaluation. For 6 years, the patient has been treated for UC with combinations of steroids and mesalamines. He always discontinued therapy due to what he perceived were adverse drug reactions: pruritic blisters affecting his chest, abdomen, back, and arms. He describes multiple bloody bowel movements, pain with defecation, and a 12-pound weight loss over 2 months. His most recent colonoscopy revealed pancolitis. He has never been in clinical remission. He was referred to a dermatologist for evaluation of his skin lesions, to determine if the dermatitis reaction was a true adverse drug reaction. Hyperpigmentation on his chest and back were noted and appeared to be due to a resolved bullous dermatosis (Figure 1). Skin biopsies identified Linear IgA bullous dermatosis. Dapsone 100 mg daily resulted in resolution of the rash. He was subsequently treated for UC with infliximab and achieved a deep remission. Persistently elevated alkaline phosphatase was investigated with MRI. This revealed intrahepatic duct dilation, and liver biopsy confirmed comorbid PSC. DISCUSSION: LABD is defined by three criteria: vesicles/bullae, subepidermal vesicles with a neutrophil predominance and the linear pattern of IgA basement membrane deposits. This case identifies a rare extra-intestinal skin manifestation of IBD. The exact pathophysiology of the association between these distinct, yet inter-related diseases, has yet to be elucidated.

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