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Author: Vanessa Ngan, Staff Writer, 2003.

Angiokeratoma — codes and concepts

What is an angiokeratoma?

An angiokeratoma is a small red to purple papule. It may have a rough scaly surface. It is composed of surface blood vessels (dilated capillaries). Often unnoticed, an angiokeratoma may become crusty and bleed if accidentally scratched or damaged, or a harmless clot may form in the lesion (thrombosis), changing the colour to dark purple or black overnight.

There are several types of angiokeratomas:

Sporadic angiokeratoma

  • Solitary lesions
  • Common in those over 40 years
Sporadic angiokeratoma

Angiokeratoma of Mibelli

  • Multiple red to blue, dry to warty papules on fingers and toes, hands and feet, or less often knees, elbows, breasts
  • Lesions appear in childhood or adolescent
  • May be familial
  • More common in females than in males
  • Lesions may bleed with minor trauma
  • Associated with chilblains
Angiokeratoma of Mibelli

Angiokeratoma of Fordyce

  • Most commonly found on the scrotum. Also found on the shaft of the penis, labia majora of the vulva, inner thigh and lower abdomen
  • Most prevalent in those over 40 years
  • Men are more often affected than women
  • Can present as a single lesion or multiple lesions (> 100)
  • Lesions are small, red and less scaly in younger patients whilst in older patients, they tend to be larger, blue/black and with overlying scales
  • Usually symptomless and may only be noticed when they bleed after scratching or intercourse
Angiokeratoma of Fordyce

Angiokeratoma circumscriptum

  • Rare birthmark (vascular malformation). Maybe present at birth but can occur later in childhood or adulthood
  • Females are more affected than men 3:1
  • A cluster of lesions on a small area of the leg or trunk
  • Over time lesions may darken in colour and change shape and size
Angiokeratoma circumscriptum

Fabry disease (angiokeratoma corporis diffusum)

  • Fabry disease is a rare serious inherited disorder caused by a deficiency of an alpha-galactosidase enzyme, ceramide trihexosidase
  • Excessive quantities of glycosphingolipids are deposited in blood vessels and internal organs
  • More severe in males than females
  • Angiokeratomas are widespread, most numerous on lower trunk and groin area
  • May present with fever and painful hands and feet
  • May result in corneal opacities, kidney failure, heart failure, strokes, arthritis, colitis and many other problems.
Angiokeratoma corporis diffusum

See more images of angiokeratomas.

What causes angiokeratomas and who gets them?

Apart from Fabry disease, which is caused by a genetic defect, the cause of other angiokeratomas is unknown. 

See also, angiokeratoma pathology.

What is the treatment for angiokeratomas?

Angiokeratomas are harmless surface vascular lesions that can usually be left alone. As the black spots sometimes resemble melanoma, a skin biopsy may be performed to rule out malignancy and allay any fears.

If the bleeding becomes a concern or treatment is requested for cosmetic purposes, they can be removed. Surgical options include excision, laser therapy, cryotherapy or electrocautery.

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