Several autosomal dominant skin diseases may manifest cutaneous mosaicism. Two types of segmental arrangement can be distinguished: type 1 is characterized by segmental lesions with similar severity to that observed in the diffuse phenotype, the remaining skin being normal; type 2 is characterized by segmental lesions showing a major degree of severity and milder lesions diffusely arranged. Multiple glomus tumours have recently been included in the group of genodermatoses showing type 2 segmental involvement. A family with 2 cases of multiple glomangiomas arranged in a segmental fashion is reported. A 12-year-old girl presented multiple nodular glomangiomas on her right buttock and thigh, in a band-like distribution. A sister of her paternal grandfather showed plaque-like multiple glomangiomas on her left thigh and various glomangiomas on her right buttock and arm. No other family members were known to be affected. Two new cases of familial segmental multiple glomangiomas are reported, with the particularity that one of these exhibited type 2 segmental manifestation and the other type 1 from a clinical point of view.
Copyright 2000 S. Karger AG, Basel.