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Angiokeratoma Corporis Circumscriptum (Fabry)


Vascular malformation characterized by dilation of superficial vessels of the dermis and hyperkeratosis of the overlying epidermis. Lesions present as solitary hyperkeratotic papules or plaques with an uneven verrucous surface, ranging in colour from deep-red to blue-black. Typical areas of involvement are the thighs, lower legs, and buttocks, but lesions may also occur elsewhere. The lesions may be present from birth or develop later on. There is no tendency to spontaneous involution.




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