Abstract
Angiokeratoma circumscriptum neviforme: extensive involvement of lower limb
Highlights
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
Localized angiokeratomas are classified as angiokeratoma of Fordyce, located in the genital region, angiokeratoma of Mibelli, located on acral parts following exposure to cold, solitary and multiple angiokeratoma, and angiokeratoma circumscriptum, which is rare
angiokeratoma circumscriptum neviforme (ACN) presents itself as bluish-black papules and nodules grouped to form a verrucous and hyperkeratotic plaque
Summary
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]. The localized forms have been classified into solitary angiokeratoma, angiokeratoma of Fordyce, angiokeratoma of Mibelli, and angiokeratoma circumscriptum neviforme (ACN). 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]
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