Angiokeratomas are telangiectasias with keratotic elements. They present in different clinical scenarios including (a) solitary or multiple angiokeratomas occurring predominantly on lower extremities; (b) angiokeratoma of Fordyce affecting the scrotum and the vulva; (c) angiokeratoma of Mibelli, an autosomal dominant disorder affecting dorsum of hands and feet, elbows, and knees; (d) angiokeratoma corporis diffusum associated with Fabry’s disease, an X-linked recessive disorder characterized by α-galactosidase-A deficiency and affecting the lower abdomen, buttocks, and genitalia; and (e) angiokeratoma circumscriptum usually grouped on one extremity.
Age: solitary or multiple angiokeratomas usually affect young adults, angiokeratomas of Fordyce affect middle-aged and elderly individuals. Angiokeratoma of Mibelli and angiokeratoma circumscriptum are usually diagnosed in childhood.
Sex: angiokeratoma of Mibelli and angiokeratoma circumscriptum exhibit female predominance. Otherwise, there is no sex predisposition.
Red to violaceous, well-circumscribed hyperkeratotic papules and plaques.
Solitary lesions can be mistaken for melanoma, acquired hemangioma, lymphangioma, seborrheic keratosis, and warts.
Marked dilated, thin-walled blood vessels in the papillary dermis, associated with an overlying acanthotic hyperkeratotic epidermis.
Management of angiokeratomas remains a challenge. Many modalities have been reported in the literature with variable success. Treatment modalities include
(A) Angiokeratomas on the abdomen of a young patient. (B) Angiokeratoma imaged through an epiluminescence microscope (DermLite)
Angiokeratoma on the left thigh resistant to multiple treatments with pulsed dye laser
(A) Biopsy-proven angiokeratoma on the thigh of a young child. (B) Some resolution after one treatment with pulsed dye laser at a wavelength of 595 nm with a 10-mm spot, pulse duration of 1.5 ms, a fluence of 7.5 J/cm2, and DCD 30/20