Angiokeratoma circumscriptum neviforme: An entity, few and far between

Abstract

Angiokeratomas are a group of vascular ectasias that involve the papillary dermis and may produce papillomatosis, acanthosis and hyperkeratosis of the epidermis. Angiokeratoma circumscriptum is the least common variant among many types. Angiokeratoma circumscriptum neviforme is a still rarer variety of angiokeratoma, which is classically seen at birth. We report here a case of congenital, linear, unilateral, verrucous plaques on the leg of a young girl, diagnosed as angiokeratoma circumscriptum neviforme (ACN).

Angiokeratoma circumscriptum

“…The mechanism for development of AKC is unknown. Several etiologic factors have been proposed, such as congenital causes, chronic trauma, tissue asphyxia, pregnancy, and subcutaneous hematomas [7].…”

“…Clinically, AKC presents as dark-red to blue-black papules or nodules that often become confluent [7,8]. Commonly it is present on the lower limbs since birth in a segmental and unilateral distribution [1,7]. The initial presentation may be of multiple red macules like a capillary malformation.…”

“…The initial presentation may be of multiple red macules like a capillary malformation. Then, within a few years, they evolve into papules and nodules; the surface of the lesion becomes hyperkeratotic and verrucous [7,8]. The lesions do not show propensity towards spontaneous regression.…”

“…The entity is benign and it is not frequently associated with systemic disease. There are reports of coexistence of AKC with Klippel-Trenaunay, Cobb syndrome, nevus flammeus, cavernous hemangioma, traumatic arteriovenous fistula, and angiokeratoma of Fordyce [3,7].…”

A rare clinic type of angiokeratoma: angiokeratoma circumscriptum naeviforme

“…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 [7]. The main pathogenetic mechanism is, ectatic dilatation of capillaries in the papillary dermis with resultant secondary epidermal changes of hyperkeratosis, acanthosis [5].…”