Angiokeratoma circumscriptum neviforme: Revisiting a rare entity

Angiokeratoma circumscriptum neviforme (ACN) is an extremely rare cutaneous disorder, occurring due to congenital malformation of the papillary dermal blood vessels. Clinically, it presents as well circumscribed, hyperkeratotic papules, nodules or plaques, often resembling malignant tumors like malignant melanoma or pigmented basal cell carcinoma. Thus, this uncommon but significant lesion should be diagnosed at the earliest, to avoid unnecessary confusion. We report here a case of congenital, linear, unilateral, verrucous plaques on the leg of a young girl, diagnosed as ACN.