Dermatology/Angiokeratoma

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Question
I have multiple angiokeratomas all over my body.  They are mainly located on my thighs and groin (private area), but are also located on my gums, feets, fingers, elbows, waist, etc.  When I was younger, I was tested for Fabry's Disease and the results came back negative.  The angiokeratomas are getting worse and more abundant.  Are there any other conditions that I should be concerned about and even though I was cleared of Fabry's Disease when I was youner, should I get tested again?  I am honestly at a loss of what to do.  My dermatologist tells me that my only choice is laser, but I am more concerned about why I am getting them.  They have been affecting me more and more because the ones covering my heel are very painful and even the laser does not get rid of them.  Please help and point me in the right direction.

Answer
Angiokeratomas are small dark red to purple raised spots. They may also have a rough scaly surface. They are composed of surface blood vessels (dilated capillaries). Often unnoticed, they 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
Angiokeratoma of Fordyce
Angiokeratoma circumscriptum
Fabry syndrome (angiokeratoma corporis diffusum)
What causes angiokeratomas and who gets them?

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

Distinguishing features

 
Sporadic angiokeratoma     
Scrotal angiokeratomas
(Fordyce)     
Vulvar angiokeratomas
(Fordyce)

Angiokeratoma circumscriptum
Angiokeratomas
Type of angiokeratoma    Description
Sporadic angiokeratoma   
Solitary lesions
Common in those over 40 years
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
May be 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 circumscriptum   
Rare birthmark (vascular malformation). May be present at birth but can occur later in childhood or adulthood
Females are more affected than men 3:1
Cluster of lesions on a small area of the leg or trunk
Over time lesions may darken in colour and change shape and size
Fabry syndrome (angiokeratoma corporis diffusum)   
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 & feet
May result in corneal opacities, kidney failure, heart failure, strokes, arthritis, colitis & many other problems.
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 bleeding becomes a concern or treatment is requested for cosmetic purposes, they can be removed. Surgical options include excision, laser therapy, cryotherapy or electrocautery.

Dermatology

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Michael S. Fisher, <B>Ph.D., M.D.</B>

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