Treatment of Refractory Pruritic Dermatitis in the Setting of Primary Biliary Cholangitis and CREST Syndrome With Upadacitinib

Primary biliary cholangitis (PBC) can present with overlapping features of limited systemic sclerosis, commonly known as calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST). Here, we discuss the case of a PBC patient with CREST who experienced treatment-resistant and progressively worsening pruritic dermatitis that responded to upadacitinib with associated improvement of her liver enzymes and symptoms. This is the first documented report of a Janus kinase (JAK) inhibitor used to treat cutaneous symptoms in the setting of an autoimmune liver disease.