Angiokeratoma circumscriptum of the oral cavity.

Sir, The term angiokeratoma is applied to several distinct unrelated conditions with cutaneous vascular lesions, the histology of which shows super¢cial dermal vascular ectasia with overlying hyperkeratosis of the epidermis. Di¡erent types of angiokeratomas have been described. They are (i) the generalized systemic type ^ angiokeratoma corporis di¡usum of Fabry; (ii) the bilateral form occurring in the dorsa of ¢ngers and toes ^ angiokeratoma of Mibelli; (iii) the localized scrotal form ^ angiokeratoma of Fordyce; (iv) the usually solitary papular angiokertoma; and (v) the multiple papular and plaque like ^ angiokeratoma circumscriptum (1). Though all types di¡er clinically they share similar histological features. They are characterized by hyperkeratosis, acanthosis and dilated capillaries in the papillary dermis, partly or completely enclosed by the papillomatous epidermis. Organized or organizing thrombi are occasionally observed within the dilated capillaries. Moreover, in Fabry's disease and fucosidosis, swollen and vacuolated endothelial cells are also identi¢ed in the vascular spaces. Angiokeratoma circumscriptum presents as multiple purple papules that later become verrucous and may coalesce to form plaques. The lesions are situated typically on the lower leg or foot but can occur on the thigh, buttock or occasionally elsewhere. In this report, we describe a case of angiokeratoma circumscriptum of the tongue that we believe is probably the ¢rst case reported in the literature.