A case of verrucous venous malformation and challenges associated with treatment

Verrucous venous malformation (VVM) is a rare congenital vascular malformation that initially presents as blue-red papules and plaques, and may progress to form hyperkeratotic and verrucous lesions. We report the case of a 20-year-old woman with congenital VVM of the left lower limb, whereby painful, crusted and easily bleeding plaques caused anxiety and functional impairment. These lesions remained static during childhood but enlarged significantly at puberty. Clinical examination revealed multiple hyperkeratotic and exophytic plaques and nodules across a 10 × 10 cm area of the left thigh and knee. Histopathology demonstrated epidermal hyperkeratosis with dilated vessels extending into the reticular dermis and subcutis. Immunohistochemistry was positive for glucose transporter-1 protein and Wilms tumour-1, confirming VVM and excluding angiokeratoma circumscriptum naeviforme. Magnetic resonance imaging demonstrated no deep structure involvement. A multidisciplinary team review recommended curettage and cautery, which resulted in rapid recurrence. Staged surgical excision has therefore been initiated with satisfactory recovery and lack of lesion recurrence at excision site. Further surgery to remove the remainder of the lesions is planned, with consideration of wider excision and skin grafting. This case also underscores the importance of early histopathological diagnosis to guide appropriate management, and highlights sirolimus as a promising adjunct or alternative therapeutic option in VVM.