Juvenile xanthogranuloma, a cutaneous lipidosis, is generally considered an isolated, insignificant lesion, although it has been associated with neurofibromatosis, and occasionally with systemic involvement.
This is the first case we are aware of in which this lesion was found in a patient with Niemann-Pick disease, a fatal lipidosis, and we urge a careful examination of patients exhibiting xanthogranulomas.
Sibulkin D, Olichney JJ. Juvenile Xanthogranuloma in a Patient With Niemann-Pick Disease. Arch Dermatol. 1973;108(6):829-831. doi:10.1001/archderm.1973.01620270051015