Verrucous hemangioma with angiokeratoma-like features

Verrucous hemangioma (VH) is a rare vascular malformation showing characteristic clinico-pathologic features. Since its formal individualization in 1967, little has been written on this entity. Here we report a case of extensive VH involving the whole length of the right lower limb of a 39-year old man. Clinically it consisted of numerous bluish-red, well-demarcated plaques and nodules with keratotic surface arranged in a linear fashion. On the lower third of the leg also an irregular shaped ulcer with red base and prominent definite borders was present. Differential diagnosis included angiokeratoma circumscriptum neviforme (ACN) and Cobb-Syndrome mainly; also lymphangioma circumscriptum and angioma serpiginosum were considered. Histologic findings revealed hyperkeratosis, acanthosis and papillomatosis in the epidermis. In the superficial and reticular dermis and partly in the subcutaneous tissue vascular dilatation and proliferation were observed. Finally, immunohistochemical findings of endothelial cells staining with factor VIII-related antigen and CD 34, confirmed the vascular nature of the lesion.