Five clinical types of angiokeratoma are recognized (refer to individual subtypes for more detailed information and images):
- Solitary or multiple angiokeratoma – Bluish to black verrucous plaques or nodules that develop on the lower extremities of adults.
- Angiokeratoma of Mibelli – Single or multiple, punctate, pinkish macule(s) arise on the dorsum of fingers and toes in adolescence. Over time, some lesions become dark blue-red, papular, hyperkeratotic, and even verrucous. Papules may form grape-like clusters as they evolve. Perniosis or acrocyanosis may be associated. Familial cases have been reported.
- Angiokeratoma corporis diffusum – Multiple tiny red macules and papules are scattered predominantly on the lower torso and thighs or these may be more widespread. Angiokeratoma corporis diffusum is seen in Fabry disease, a rare X-linked recessive lysosomal storage disorder, as well as other rare inherited metabolic disorders such as fucosidosis, sialidosis, Kanzaki disease, beta mannosidosis, aspartylglycosaminuria, galactosialidosis, and GM1 gangliosidosis.
- Angiokeratoma of Fordyce – 2-3 mm, smooth, red or violaceous papules are seen on the scrotum or vulva, typically in older individuals. Occasionally these may be larger (up to 5 mm) or warty.
- Angiokeratoma circumscriptum – One or more plaques of blue-red papules with a verrucous surface develop during childhood, most often on the lower limb. Angiokeratoma circumscriptum has been associated with Klippel-Trenaunay-Weber syndrome (osteohypertrophy of a limb), Cobb syndrome, port-wine stain, cavernous hemangiomas, and arteriovenous fistulas.
