Type 2 segmental manifestation of congenital multiple glomangiomas

Congenital multiple glomus tumors are extremely rare, and less than 20 cases have been well documented. We report an uncommon case of congenital multiple glomangiomas with a segmental manifestation in a 9-year-old girl. Since birth, the child had presented asymptomatic angiomatous macules arranged in a segmental pattern on the neck and trunk. During a follow-up period of 9 years, disseminated smaller papulonodular lesions developed on both arms and the left leg with a segmental distribution. Histopathology of congenital and acquired lesions confirmed the diagnosis of glomangiomas. The family history was negative for glomus tumors. This type of presentation supports the recently described type 2 segmental manifestation of multiple glomus tumors, with a segmental involvement of congenital early developing lesions.