Variant Distribution of Skin Lesions in Angiokeratoma Circumscriptum: Report of an Untypical Case

Huan Wang, Yan Guo Zhang, Yao Qing Yu


Angiokeratoma circumscriptum is one of rare skin lesions mainly manifested in hyperkeratotic papules or nodules occurring frequently unilaterally on the foot or leg and most characteristically takes the form of an extensive vascular plaque. Herein, we report an untypical case of angiokeratoma circumscriptum. Different from the typical form of localized extensive vascular plaque and unilaterally location of the foot or distal aspect of the leg, the skin lesions of present case initiated in the left elbow and recently progressed to ipsilateral arm and trunk. In addition, the skin lesions are varied from multiple, discrete papules to confluent, protruding verrucous plaques. The physical and histopathological findings are consistent with a diagnosis of angiokeratoma circumscriptum. The untypical distribution of skin lesions indicates that the forms of angiokeratoma circumscriptum may be varient in clinic. Since present case shares similar appearances in cutaneous vascular lesions (angiokeratomas) with systemic angiokeratoma corporis diffusum (Fabry disease), the difference between the two entities is also discussed.

J Med Cases. 2012;3(5):297-299


Angiokeratoma circumscriptum; Fabry disease

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