Glomuvenous malformations (GVM), previously known as glomangiomas, are an uncommon subset of venous malformation (VM), characterized by distinct histopathology, clinical presentation and association with loss-of-function mutations in the glomulin gene… Click to show full abstract
Glomuvenous malformations (GVM), previously known as glomangiomas, are an uncommon subset of venous malformation (VM), characterized by distinct histopathology, clinical presentation and association with loss-of-function mutations in the glomulin gene (GLMN) [1]. Classically, they occur as painful, bluish solitary papules, widely scattered nodules or segmental plaques that are frequently hyperkeratotic and may assume a cobblestone-like appearance. In contrast to classic VM, GVM are often present at birth, and increase in size slowly during childhood. They rarely affect the mucosa, viscera or joints and a positive family history is present in approximately two thirds of the patients [2]. While skin biopsy is not always required for diagnosis, histopathology of GVM demonstrates venous-like channels surrounded by glomus cells, which stain positive for vimentin and -smooth muscle actin and negative for desmin [1, 3]. Treatment options in selected cases include sclerotherapy, laser therapy and surgical excision [1].
               
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