Angiokeratoma Circumscriptum Naeviforme and Klippel–Trenaunay Syndrome: A Diagnostic Enigma

Angiokeratoma circumscriptum naeviforme (ACN) is a rare subtype of angiokeratoma, characterized by capillary ectasias in the papillary dermis and presenting as hyperkeratotic, bluish-red plaques primarily along Blaschko’s lines on the lower limbs. Although usually isolated, ACN can rarely be associated with vascular disorders such as Klippel–Trenaunay syndrome (KTS), Cobb’s syndrome, and arteriovenous fistulas. KTS, defined by the presence of at least two features of the triad – capillary malformation, varicose veins, and soft-tissue hypertrophy – typically includes a port wine stain (PWS) as its most common skin manifestation. Here, we report two unique cases of young females with ACN and features of KTS but without PWS, an exceedingly rare presentation. Our findings suggest that ACN, when co-occurring with persistent embryonic veins, could indicate a variant of KTS and expand the spectrum of KTS-associated vascular anomalies.