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Angiokeratomas are characterized by ectasia of superficial or papillary vessels with overlying hyperkeratosis [1], first described by Mibelli in 1889 and termed angiokeratoma circumscriptum by Dammert. The mechanism of development remains unknown [2]. Angiokeratomas may be localized or generalized. The localized forms have been classified into solitary angiokeratoma, angiokeratoma of Fordyce, angiokeratoma of Mibelli, and angiokeratoma circumscriptum neviforme (ACN). Among these, ACN is the rarest, characterized by the presence of hyperkeratotic papules developing into verrucous bluish-black plaques commonly on the lower limbs in a segmental distribution. A lack of spontaneous regression makes the elimination of ACN imperative by the use of an appropriate modality (diathermy, curettage, electrocautery, cryosurgery, deep excision followed by grafting, CO2/argon/KTP laser) [2,3].

Angiokeratoma circumscriptum neviforme: extensive involvement of lower limb