Xanthoma Striatum Palmare Associated With Nonsyndromic Paucity of the Interlobular Bile Ducts

A 1-year-old adopted boy presented to the dermatology clinic for evaluation of pruritus and yellow papules on his palms. The patient was born at term without complications. The medical history of his biological parents was unknown. He developed jaundice at 10 weeks of age. A liver biopsy revealed pathologic findings worrisome for biliary atresia, and a Kasai procedure (hepatoportoenterostomy) was performed. Unfortunately, the Kasai procedure did not result in clinical improvement. His degree of hypercholesterolemia also was inconsistent with a diagnosis of biliary atresia, leading to an evaluation for Alagille syndrome.