Sir,
Angiokeratomas are verrucous, hyperkeratotic, red-black, well-demarcated papules and plaques consisting of vascular enlargement and hyperkeratosis [
A 43-year-old man presented with a red-black lineer eruption on his left leg which had been present since infancy. He gave a history of several episodes of mild bleeding from the lesions after minor trauma. There were no other local or systemic symptoms. There was no history of similar disease in his family. Dermatological examination revealed hyperkeratotic papules over dark red-black plaques on his gluteus, thigh and calf extending to the dorsum of the foot following the lines of Blaschko (
Hyperkeratotic papules over dark red-black plaques on the left gluteal region.
The verrucous nodule and hyperkeratotic patches on the pretibial region.
The lesions extending from left glutea to dorsum of the foot following the lines of Blaschko.
Hyperkeratosis and acanthosis of the stratified squamous epithelium, congestion in the papillary dermis and dilated vascular structures (HE × 100).
Angiokeratoma sircumscriptum naeviforme is the rarest reported type of angiokeratomas. The lesions of angiokeratoma sircumscriptum neviforme are typically present since birth or infancy. Women are affected more commenly than men [
Although different in terms of appearance and layout, morphological variants of angiokeratoma are histologically similar [
Although the exact reason of the formation of angiokeratomas are unknown, several causal factors, such as congenital development, pregnancy, trauma, subcutaneous hematomas, and tissue asphyxia, have all been proposed [
Our main differential diagnosis of the case was verrucous hemangioma. Verrucous hemangioma is a congenital, localized vascular malformation. Histopathologically, it is characterized by dilated capillaries and red blood cell-filled spaces extending well into the reticular dermis and subcutaneous tissue with an overlying hyperkeratotic epidermis [
There is no tendency of spontaneous regression in angiokeratoma sircumscriptum naeviforme. Patients may require the removal of the lesions for cosmetics reasons. Smaller lesions may be managed by diathermy, electrocautery, cryosurgery, currettage and surgery. Laser ablation [carbon dioxide or argon laser] may be used for larger lesions which are not suitable for surgery [
We reported here a case with congenital, linear, unilateral, verrucous plaques on the lower extremity, diagnosed as angiokeratoma circumscriptum neviforme. We report this case due to its rarity and want to emphasize that, in spite of its rarity, anigokeratoma circumscriptum should be considered in the differential diagnosis of linear verrucous lesions.