Nevus lipomatosus cutaneus superficialis of the scalp

A 12-year-old boy with nevus lipomatosus cutaneus superficialis of the scalp is described. Asymptomatic plaques made up of varying sized aggregates of elastic soft papules were observed on the occipital region. Histologic examination of a biopsy specimen revealed ectopic fat tissue in the dermis. Two types of nevus lipomatosus cutaneus superficialis can be differentiated clinically. The classic types, with multiple lesions in a zonal distribution, occur most often on the lumbar region, buttocks, and thigh. The other types, which involve a solitary papule or nodule, have no favored location. This is the first report of multiple lesions in a zonal distribution occurring characteristically on the scalp.