Angiokeratoma a is benign cutaneous lesion of capillaries, resulting in small marks of red to blue color and characterized by hyperkeratosis

Hyperkeratosis

Hyperkeratosis is thickening of the stratum corneum, often associated with a qualitative abnormality of the keratin, and also usually accompanied by an increase also in the granular layer...

.

"Angiokeratoma corporis diffusum" refers to Fabry's disease

Fabry's disease

Fabry disease is a rare X-linked recessive lysosomal storage disease, which can cause a wide range of systemic symptoms...

, but this is usually considered a distinct condition.

Presentation

Presentation includes telangiectasia

Telangiectasia

Telangiectasias /tɛ.læn.dʒiː'ɛk.teɪ.ʃi:ə/ are small dilated blood vessels near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. They can develop anywhere on the body but are commonly seen on the face around the nose, cheeks, and chin...

, acanthosis

Acanthosis

Acanthosis is diffuse epidermal hyperplasia.Acanthosis implies increased thickness of stratum spinosum. It is a disease ofthe prickle cell layer of the skin, where warts appear on the skin or inside the mouth.-References:...

, and hyperkeratosis

Hyperkeratosis

Hyperkeratosis is thickening of the stratum corneum, often associated with a qualitative abnormality of the keratin, and also usually accompanied by an increase also in the granular layer...

.

Presentation can be solitary or systemic.

Possible misdiagnosis

Due to the rarity of different types of vascular conditions, angiokeratomas may be misdiagnosed. A biopsy

Biopsy

A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...

 of the lesion can produce a more accurate diagnosis.

Histopathology

Angiokeratomas characteristically have large dilated blood vessel

Blood vessel

The blood vessels are the part of the circulatory system that transports blood throughout the body. There are three major types of blood vessels: the arteries, which carry the blood away from the heart; the capillaries, which enable the actual exchange of water and chemicals between the blood and...

s in the superficial dermis

Dermis

The dermis is a layer of skin between the epidermis and subcutaneous tissues, and is composed of two layers, the papillary and reticular dermis...

 and hyperkeratosis

Hyperkeratosis

Hyperkeratosis is thickening of the stratum corneum, often associated with a qualitative abnormality of the keratin, and also usually accompanied by an increase also in the granular layer...

(overlying the dilated vessels).

Potential complications

In some instances nodular angiokeratomas can produce necrotic tissue and valleys that can harbor fungal, bacterial and viral infections. Infections can include staphylococcus. If the lesion becomes painful, begins draining fluids or pus, or begins to smell, consult a physician. In these instance a doctor may recommend excision and grafting.

Treatment

Outpatient treatments such as interventional radiology, lasers, and physical therapy are employed to reduce the severity of the vascular lesions. However, in some cases lasers have caused a reaction in the tissue causing it to expand and become exposed to infection. Excision and grafting may be necessary to remove the lesion. Recovery time on such an operation ranges from 3 to 12 weeks depending on location of the graft, healing time and the possibility of complications.

External links

• Cincinnati Children's Center for Hemangioma and Vascular Malformations http://www.cincinnatichildrens.org/svc/alpha/h/vascular/default.htm