Facial "glomangiomas": Large facial venous malformations with glomus cells

Background: "Glomangiomas" are benign cutaneous vascular lesions consisting of convoluted, abnormally formed venous channels lined by cuboidal and ova l epithelioid, a-actin-positive, glomus cells. Three different clinical var iants of glomangioma have been recognized: solitary, multiple, and nodular, or plaquelike. Inheritable forms are common. Objective: We describe in 7 patients (2 of them having a familial glomangio matosis) the rare facial location of glomangiomas to differentiate this typ e from common facial venous malformation (VM). Methods: We analyzed clinical data (photographs), course, investigations (c omputed tomographic scans in 4 patients, magnetic resonance imaging in 6, a rteriography in 2. direct puncture phlebography in 4, and pathologic examin ations in all, and outcome with treatment. Results: Lesions were soft, composed of multiple nodules, confluent and pla quelike, deep blue or blue-to-purple, sometimes sagging, one-sided in a che ek, extending to the lips in 5 patients, to the chin in 4, and to the lower eyelid In 4. They were poorly compressible, a finding different from commo n facial VMs. In a young man extensive back involvement was associated. Amo ng radiologic investigations, only magnetic resonance imaging after gadolin ium enhancement offered some differential features with common VMs. However , histopathologic examination clarified the differential diagnosis: althoug h the large tortuous venous channels were reminiscent of capillary-venous m alformation, in many vessels the walls contained one or several rows of glo mus cells. Conclusion: Multiple plaquelike facial "glomangiomas" mimic a common venous malformation because of their blue hue. However, with experience, one can clinically recognize them, and their pathologic aspect is distinctive. Mana gement should differ slightly from that For common facial I because sclerot herapy has proven to be less effective. Therefore surgical treatment is the only helpful therapeutic option.