Linear IgA Bullous Dermatosis Preceding the Diagnosis of Primary Sclerosing Cholangitis and Ulcerative Colitis: A Case Report

Am J Dermatopathol. 2019 Jul;41(7):498-501. doi: 10.1097/DAD.0000000000001313.


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.

Publication types

  • Case Reports

MeSH terms

  • Cholangitis, Sclerosing / complications*
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / drug therapy
  • Colitis, Ulcerative / complications*
  • Colitis, Ulcerative / diagnosis
  • Colitis, Ulcerative / drug therapy
  • Humans
  • Linear IgA Bullous Dermatosis / complications*
  • Linear IgA Bullous Dermatosis / diagnosis
  • Linear IgA Bullous Dermatosis / drug therapy
  • Male
  • Young Adult