Background: In various autosomal dominant skin disorders, segmental forms reflecting mosaicism have been reported. Recently, two different types of mosaic manifestation have been delineated. Type 1 reflects heterozygosity for the underlying mutation and shows a degree of severity as observed in the corresponding nonmosaic phenotype. Type 2 originates from loss of heterozygosity, shows an excessively severe involvement and is usually superimposed on the disseminated lesions of the ordinary trait. Objective: We wanted to exemplify further the proposed rule of dichotomy. Methods: We have screened the literature on multiple glomus tumors, a trait that follows an autosomal dominant mode of transmission. Results: We found 5 cases of multiple glomus tumors suggesting a type 2 segmental involvement. In all of these cases, a unilateral band-like or patchy arrangement of excessively pronounced glomus tumors was associated with disseminated lesions corresponding to the ordinary phenotype, and in 3 cases other family members were affected with disseminated glomus tumors. The unilateral agminated lesions were reported to be present in early childhood, whereas the disseminated lesions appeared later. Conclusion: Multiple glomus tumors can be added to the list of autosomal dominant skin disorders that may show a type 2 segmental involvement.

Keywords:
Glomus tumors, Glomangioma, Mosaicism, Loss of heterozygosity, Type 2 segmental involvement
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