Management of Refractory Pruritus in Alagille Syndrome with Dupilumab Treatment: Case Report

Alagille syndrome (ALGS) is an autosomal dominant multisystem disorder with a reported incidence of 1:30,000 live births. ALGS-associated cholestasis presents in infancy and manifests with severe pruritus often leading to scratching, skin scarring, and resulting lichenoid processes. In fact, pruritus has been considered one of the most troublesome symptoms in this syndrome. Medical management for pruritus secondary to ALGS remains mostly targeted on supportive care with a variety of options including rifampin, ursodiol, and ileal bile acid transport inhibitors with promising results.