Glomuvenous Malformation (Glomangioma) and Venous Malformation

• DOI: 10.1001/archderm.140.8.971
• Publication Date: 2004-08-16T20:23:41Z
• Authors: Laurence M. Boon; John B. Mulliken; Odile Enjolras; Miikka Vikkula
• Keywords: Venous malformation; Vascular malformation; Klippel-Trenaunay Syndrome

To develop clinical criteria that permit distinction between inherited glomuvenous malformation (GVM), known as glomangioma, and cutaneomucosal venous to test these on sporadic lesions.Clinical data were compiled for 1685 patients with or cutaneous anomalies. Based a cohort of mutation in the TIE2 glomulin gene histologic diagnosis, we defined GVM malformation. We then applied cases blinded manner genetically histologically confirmed this diagnosis whenever possible.Glomuvenous malformations accounted 5.1% anomalies frequently (63.8%), whereas rarely familial (1.2%). Glomuvenous nodular scattered, plaque-like segmental, color varying from pink purplish dark blue, most (VMs) soft, often localized vascular lesions. mainly located extremities involved skin subcutis, VMs commonly affected muscles joints (P<.001). had distinct raised, hyperkeratotic cobblestone-like appearance could not be completely emptied by compression, unlike VMs. painful awakening, after activity, hormonal changes. Elastic compressive garments aggravated pain GVMs, contrast VMs.This large series superficial established features distinguish GVMs. This differential is essential, outcome treatment GVMs differ.