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Linear IgA Bullous Dermatosis Preceding the Diagnosis of Primary Sclerosing Cholangitis and Ulcerative Colitis

A Case Report

Humphrey, Victoria S. BS*; Lee, Jonathan J. MD; Supakorndej, Teerawit MD, PhD; Malik, Shahid M. MD; Huen, Arthur C. MD, PhD; Jaroslaw, Jedrych MD

The American Journal of Dermatopathology: July 2019 - Volume 41 - Issue 7 - p 498–501
doi: 10.1097/DAD.0000000000001313
Brief Report
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Abstract: Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder seen in the pediatric and adult populations that is often linked to a medication, infection, or underlying gastrointestinal, hepatobiliary, or autoimmune disease. In this study, we describe the case of a 23-year-old white man whose presentation and diagnosis of LABD ultimately led to the discovery of underlying primary sclerosing cholangitis (PSC) and ulcerative colitis (UC). His dermatitis resolved with topical steroids and dapsone, and he is undergoing systemic treatment for his UC and PSC. This exceptional case further validates the association between LABD with UC, strengthens that with PSC, and underscores the importance of alerting clinicians to consider conducting a systemic workup in addition to thorough medication history on making the diagnosis of LABD.

*University of Pittsburgh School of Medicine, Pittsburgh, PA;

Departments of Dermatology; and

Gastroenterology, Hepatology, and Nutrition, University of Pittsburgh Medical Center, Pittsburgh, PA.

Correspondence: Jaroslaw Jedrych, MD, PhD, UPMC Dermatology, 3708 Fifth Avenue Fifth Floor, Suite 500.68, Pittsburgh, PA 15213 (e-mail: jedrychj@upmc.edu).

The authors declare no conflicts of interest.

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