Angiokeratoma is benign cutaneous injury of capillaries, resulting in small lesions on the skin, typically having a red to blue color. ICD-10 code: D23.9
Fabry's disease manifests in boys during adolescence.
Angiokeratoma of Mibelli (AKM) typically develops between the ages of 10 and 15.
Scrotal and vulvar angiokeratoma (Angiokeratoma of Fordyce) develops between the ages of 16 and 70.
Classification:- Solitary angiokeratoma
- Angiokeratoma of Mibelli (AKM)
- Scrotal and vulvar angiokeratoma (Angiokeratoma of Fordyce)
- Diffuse angiokeratoma of the trunk (Fabry's disease)
Diffuse angiokeratoma of the trunk is a lysosomal storage disorder inherited in an X-linked recessive manner. Accumulation of glycolipids or sphingolipids occurs in the skin and internal organs due to deficiency of the lysosomal enzyme alpha-galactosidase A (ceramidetrihexosidase). Diffuse angiokeratoma has also been observed in fucosidosis, sialidosis, mannose-6-phosphatase deficiency, as well as without apparent metabolic disturbances.
Angiokeratoma of Mibelli is inherited in an autosomal dominant manner in families with a history of frostbite.
No specific etiology has been established for limited angiokeratoma and scrotal/vulvar angiokeratoma.Some forms of angiokeratomas may be present from birth or develop during the first decades of life, initially appearing as grouped red papules that eventually form a plaque with a verrucous surface.
Diffuse angiokeratoma of the trunk is characterized by the presence of hundreds of 1-2 mm red micropapules covering the skin of the lower extremities and lower trunk.
Angiokeratoma of Mibelli shows numerous round red papules 1-5 mm in diameter with superficial hyperkeratosis on the dorsal surfaces of the hands, feet, and fingers.
In the scrotal form (angiokeratoma of Fordyce), dark red papules are localized on the skin of the scrotum and vulva.- Determination of alpha-galactosidase levels.
- Biopsy of the lesion.
- Urinalysis
- Lichen planus
- Melanoma
- Targetoid hemociderotic hemangioma
- Trombosed angiomas
For the solitary form, surgical excision or laser ablation is performed. The diffuse form is treated symptomatically, including analgesics, hemodialysis, and kidney transplantation, depending on the patient's condition.
The prognosis is favorable for limited forms, but uncertain for systemic involvement. Complications of the disease are related to internal organ involvement (left ventricular hypertrophy, episodic fever, renal dysfunction, and pain).