CASE REPORT Xanthoma Disseminatum of the Central Nervous System and Cranium

SUMMARY: Xanthoma disseminatum is a rare normolipemic histiocytic disorder of non-Langerhans cell origin. It is a chronic systemic disease with a benign course, characterized by disseminated, yellow-orange–colored papules on the face, flexures, and mucosal membranes. We report 3 patients with xanthoma disseminatum, who presented primarily with central nervous system disease and a multitude of imaging findings throughout the craniospinal axis. Xanthoma disseminatum affects different age populations,and cases have been reported between the ages of 5months and 70 years. The disease is more prevalent in males with a male-to-female ratio of 2:1. No typical inheritance pattern or geographic distribution is recognized.1 Three clinical variants are described on the basis of disease progression and progno-sis: a common persistent form, a less common progressive form with systemic involvement, and a rare form of self-lim-ited disease. Central nervous system (CNS) involvement is rare and usually occurs in the systemic variety.2,3 Xanthoma disseminatum is characterized by a normal lipid profile.1,4 Di