Angiokeratoma circumscriptum: a case report and review of the literature

Abstract

The term “angiokeratoma” is applied to several distinct conditions with vascular skin lesions, the histology of which shows superficial vascular ectasia and overlying hyperkeratosis.1 The angiokeratomas should be regarded as capillary vascular malformation rather than hemangiomas.1 Apart from angiokeratoma corporis diffusum (Fabry), which is a disorder of phospholipid metabolism, at least four types of purely cutaneous angiokeratoma are known: the Mibelli type, the Fordyce type, angiokeratoma circumscriptum and “solitary and multiple angiokeratoma” (Imperial and Helwig). We present here a case of angiokeratoma circumscriptum and a brief review of the literature.

Angiokeratoma circumscriptum of the buccal mucosa: a case report and literature review

“…The first case of angiokeratoma was reported by Mibelli in 1890, and the manifestation of the disorder found on fingers and toes is now known as angiokeratoma of Mibelli13. Angiokeratoma circumscriptum was first described in 1915 by Fabry as a localized lesion on a lower extremity13,14.…”

“…Angiokeratoma circumscriptum was first described in 1915 by Fabry as a localized lesion on a lower extremity13,14. It typically occurs unilaterally on the lower legs or feet, but it has also been observed on the thighs, buttocks, or elsewhere.…”

“…It typically occurs unilaterally on the lower legs or feet, but it has also been observed on the thighs, buttocks, or elsewhere. The lesions are deep red to blue-black in color and are usually elevated, warty, purplish and compressible papules13. In most cases, angiokeratoma circumscriptum is present at birth, but in some cases, it develops during childhood or adulthood4,7.…”

Angiokeratoma circumscriptum neviforme: An entity, few and far between

“…Variants of angiokeratomas include solitary and multiple angiokeratoma, angiokeratoma circumscriptum, angiokeratoma of Fordyce, angiokeratoma of Mibelli, and angiokeratoma corporis diffusum. [1] Angiokeratoma circumscriptum is the rarest variant. Clinically, it is manifested as dark red to blue-black nodules or plaques presenting unilaterally over the lower extremities.…”

Late onset palmar angiokeratoma circumscriptum: An unusual presentation

“…[2] Lesions of AC are typically situated unilaterally on the lower legs or feet, but can occur on the thighs, buttocks, or occasionally elsewhere. [3] The age of onset of AC is generally at birth. Late onset AC has been reported, however this is rare.…”