Abstract
A 2-month-old girl presented with a 9 x 2.5 cm pink and purple patch on the chest since birth. There was superimposed vascular prominence, with multiple adjacent 3-6 mm macular telangiectases. On the back were multiple blue to purple atrophic plaques with vascular prominence and surrounding telangiectases. There were no evident symptoms, and she was otherwise healthy. Family history was negative for similar lesions. Two 4mm punch biopsies were performed. Within the dermis and subcutis was a poorly circumscribed lesion composed of widely scattered, variably-sized, thin-walled blood vessels with a surrounding perivascular cuff of glomus cells. The glomus cells were positive for SMA and negative for cytokeratin AE1/AE3 and CD31. When correlated with the clinical presentation, the findings were consistent with segmental congenital plaque-like glomuvenous malformation. Glomuvenous malformations are hamartomas with a larger proportion of blood vessels and smaller proportion of glomus cells than conventional glomus tumors. Plaque-like variants are rare, typically with an onset in childhood, and may present with either aggregated blue nodules1 or atrophic, telangiectatic plaques2. This case provides additional clinicopathologic correlation of an uncommon tumor with a distinctive clinical presentation as multiple segmental lesions.
1. Souza NGA, Wedy GF, Nai GA, Morgado de Abreu MAM. Congenital plaque-like glomangioma: report of two cases. An Bras Dermatol. 2017;92(5 Suppl 1): 43-6
2. Vargas-Navia N, Baselga E, Munoz- ˜ Garza FZ, Puig L. Malformación glomovenosa congénita en placas: 11 anos ˜ de seguimiento y respuesta al tratamiento con láser combinado PDL/Nd: YAG. Actas Dermosifiliogr. 2017;108:72---74.