Congenital linear steatocystoma multiplex of the nose

Abstract

We describe a case of steatocystoma multiplex with an unusual clinical manifestation. A 5-year-old girl had 8 papulonodular lesions arranged in a linear fashion on the left side of the nose. These had been present at birth. Histologically the lesions showed the typical features of steatocystoma multiplex. There was no family history of similar lesions. This is the second case of linear steatocystoma multiplex, a rare variant of steatocystoma multiplex.