Angiokeratoma

Key Points
Skin growths which may affect any area of the body
Exact cause of angiokeratomas is unknown in most cases, excepting Fabry’s syndrome which is due to a genetic mutation and usually consists of multiple angiokeratomas
Consists of elevated lesions which are dark blue or black in color and may be accompanied by scaling

Antiokeratomas are skin growths which may affect any area of the body. The condition consists of an elevated papule (bump) which is dark blue or black in color and may be accompanied by scaling and roughness in the overlying skin. Injury can lead to bleeding and/or crusting of lesions, or clots may form within them, darkening their color substantially. Angiokeratomas come in several forms.

Sporadic Angiokeratoma typically consists of solitary lesions, which may appear in various areas. Fordyce angiokeratomas may be solitary or occur in great numbers (100+), and are most commonly found on the scrotum in men and the labia in women. Lesions may also occur on the shaft of the penis, the inner thigh and on the lower part of the abdomen. Angiokeratoma circumscriptum is a rare vascular malformation which presents as clusters of lesions in localized area on the torso or leg, which may change shade, shape and size as time passes. Fabry’s Syndrome (Angiokeratoma corporis diffusum) is a rare genetic mutation which causes an enzyme deficiency. This leads to widespread cutaneous presentation, in addition to systemic symptoms such as eye disorders, kidney or heart failure, stroke, and arthritis.

In most cases the cause of Angiokeratomas is unknown, excepting Fabry’s Syndrome, which is caused by genetic mutation and is more severe in males. Sporadic and Fordyce Angiokeratomas typically develop after age 40, and Fordyce is far more common in men than in women. Angiokeratoma circumscriptum may present in infancy, but may also occur later in life, and is more common in women than men.

Differential Diagnosis (Other conditions with similar appearance)

Nevi, melanocytic
Cherry Hemangioma
Pyogenic granuloma
Squamous cell carcinoma
Malignant Melanoma
Blue rubber bleb nevus syndrome
Osler-Weber-Rendu syndrome

Diagnosis
Key Points
Diagnosis based on clinical exam and sometimes dermoscopy of the affected area
Skin biopsy may be performed to confirm diagnosis and rule out other conditions
Other testing may be required if Fabry’s syndrome is suspected

Angiokeratoma is diagnosed based on clinical exam in most cases. Sometimes dermoscopic examination of the affected area is done. If uncertainty exists, skin biopsy may be performed to confirm the diagnosis and to rule out similar appearing conditions. Other testing may be performed if Fabry's Syndrome is suspected, including urinalysis and other laboratory tests.

Treatment
Condition is benign, therefore treatment is not typically required
Lesions should be examined in cases which resemble malignant conditions
*If required, treatment consists of surgical removal

Angiokeratomas are benign lesions, and therefore treatment is not typically required. In cases where lesions resemble malignant conditions (Melanoma), lesions should always be biopsied. If treatment becomes necessary due to bleeding or cosmetic concerns, removal via surgical excision, laser therapies, Cryotherapy or electrocautery is the preferred method. Systemic involvement which accompanied Fabry’s Syndrome should be treated as necessary.

Written by Chris Schach