(Virtual) Glomuvenous malformation >10% BSA in 15 month old child

Abstract

Glomuvenous malformation (glomangioma), previously considered a variant of a glomus tumor, is a rare type of venous malformation (representing 5% of venous anomalies) that contains glomus cells in the walls of malformed veins (Colmenero 2021). We describe a case of an otherwise healthy 15 month old girl from Honduras with a 14 x 18 cm variegate vascular plaque on the back. The lesion contained various shades of red/blue/purple and demonstrated large caliber veins running through areas of significant atrophy. Present since birth, the patient was initiated on propranolol at 10 days old, which was stopped at 10 months old due to concern of a brown rash on her arms, without rebound. The patient was seen in our clinic for diagnosis and consideration of further treatment options. MRI of lumbar/thoracic spine +/- contrast showed a large vascular malformation confined to the dorsal skin and subcutaneous fat. A 6 mm punch biopsy from the lower median part of the largest component of the plaque was obtained. Histopathology demonstrated dilated channels, some containing erythrocytes, lined by more than one layer of monomorphous, oval-to-round blue cells. Lesional cells were positive for smooth muscle actin and negative for desmin. In the clinical context, these findings were consistent with a congenital glomuvenous malformation, plaque type. The patient was referred to a clinic specializing in vascular birthmarks.

Published in: ASDP 60th Annual Meeting

Publisher: The American Society of Dermatopathology
Date of Conference: October 2-8, 2023