Epithelioid hemangioendothelioma: A rare primary vascular tumour in skin

Epithelioid haemangioendothelioma is a distinctive tumour arising from vascular epithelium characterized histologically by epithelioid endothelial cells arranged in strands or as individual units, in a myxoid or hyalinized stroma. This tumour is more commonly seen in deeper soft tissues and involvement of the skin is very rare. Only less than 10% of cases occur primarily in the skin. We are reporting a case presented to Teaching Hospital Karapitiya with primary cutaneous epithelioid haemangioendothelioma involving the face. A 52-year-old female presented with a sclerotic skin plaque and nodules involving right side of the face for one month which developed spontaneous ulceration and bleeding. On examination revealed few violaceous firm nodules over forehead and right ear with sclerotic linear lesion over right cheek with ulceration at margin. No associated lymphadenopathy or systemic involvement. Computed tomography and MRI scan revealed an ill-defined mass in fatty tissue of skin in right cheek suggestive of skin malignancy without any internal involvement. Skin biopsy and immunohistochemistry confirmed the diagnosis of epithelioid haemangioendothelioma arising in skin and was managed with wide local excision of tumour.