Pilomatricomas in Turner Syndrome

Abstract

Pilomatricomas are benign, cutaneous tumors of the hair matrix cells, which present predominantly in childhood. Although usually an isolated finding, they have been described in association with several inherited conditions. We report a 10-year-old girl with Turner syndrome who has two pilomatricomas, including one giant tumor. In combination with a previous report of pilomatricoma in two patients with Turner syndrome, our observation indicates that it is likely a real, although uncommon, association of pilomatricomas with Turner syndrome exists. This apparent predisposition may be etiologically related to the abnormalities of hair-bearing skin observed in Turner syndrome. Doctors involved in the diagnosis and management of cutaneous masses in children should be aware of this association.