Angiokeratoma is a benign

cutaneous Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different d ...

lesion of capillaries, resulting in small marks of red to blue color and characterized by

hyperkeratosis Hyperkeratosis is thickening of the stratum corneum (the outermost layer of the epidermis, or skin), often associated with the presence of an abnormal quantity of keratin,Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2004) ''Robbins & Cotran Pathologi ...

. ''Angiokeratoma corporis diffusum'' refers to

Fabry's disease Fabry disease, also known as Anderson–Fabry disease, is a rare genetic disease that can affect many parts of the body, including the kidneys, heart, and skin. Fabry disease is one of a group of conditions known as lysosomal storage diseases. T ...

, but this is usually considered a distinct condition.

Signs and symptoms

Presentation includes

telangiectasia Telangiectasias, also known as spider veins, are small dilated blood vessels that can occur near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. These dilated blood vessels can develop anywhere on ...

acanthosis The epidermis is the outermost of the three layers that comprise the skin, the inner layers being the dermis and hypodermis. The epidermis layer provides a barrier to infection from environmental pathogens and regulates the amount of water rele ...

, and

. Presentation can be solitary or systemic. Multiple angiokeratomas, especially on the trunk in young people, are typical for

Fabry disease Fabry disease, also known as Anderson–Fabry disease, is a rare genetic disease that can affect many parts of the body, including the kidneys, heart, and skin. Fabry disease is one of a group of conditions known as lysosomal storage diseases. T ...

, genetic disorder connected with systemic complications.

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.

Pathophysiology

Histology

Angiokeratomas characteristically have large dilated

blood vessel The blood vessels are the components of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away ...

s in the superficial dermis and

(overlying the dilated vessels). File:Angiokeratoma - high mag.jpg, Scrotal angiokeratoma; visible large dilated blood vessels and

File:SkinTumors-PA090934.JPG, Scrotal angiokeratoma (Fordyce type); multiple

papule A papule is a small, well-defined bump in the skin. It may have a rounded, pointed or flat top, and may have a dip. It can appear with a stalk, be thread-like or look warty. It can be soft or firm and its surface may be rough or smooth. Some h ...

s made by dilatated

capillaries A capillary is a small blood vessel from 5 to 10 micrometres (μm) in diameter. Capillaries are composed of only the tunica intima, consisting of a thin wall of simple squamous endothelial cells. They are the smallest blood vessels in the body: ...

File:SkinTumors-PA090936.JPG, Scrotal angiokeratoma (Fordyce type); dilated cavernous capillaries,

File:SkinTumors-PA090938.JPG, Scrotal angiokeratoma (Mibelli type); blood vessels close to the epidermis File:SkinTumors-PA090939.JPG, Angiokeratoma (Mibelli type)

Diagnosis

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

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a dise ...

of the lesion can produce a more accurate diagnosis.

Classification

Angiokeratoma may be classified as: * ''Angiokeratoma of Mibelli'' (also known as "Mibelli's angiokeratoma," "Telangiectatic warts") consists of 1- to 5-mm red vascular papules, the surfaces of which become hyperkeratotic in the course of time.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. . The disease is named after Italian

dermatologist Dermatology is the branch of medicine dealing with the skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medica ...

Vittorio Mibelli Vittorio Mibelli (18 February 1860 – 26 April 1910) was an Italian dermatologist born in Portoferraio, Elba. He studied in Siena and Florence, afterwards working in Siena as a prosector at the anatomical institute and later as an assistant at t ...

(1860-1910).Mibelli's disease II
@

Who Named It ''Whonamedit?'' is an online English-language dictionary of medical eponyms and the people associated with their identification. Though it is a dictionary, many eponyms and persons are presented in extensive articles with comprehensive bibliograp ...

* ''Angiokeratoma of Fordyce'' (also known as "Angiokeratoma of the scrotum and vulva," though not to be confused with

Fordyce's spots Fordyce spots (also termed Fordyce granules)James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. . are visible sebaceous glands that are present in most individuals ...

) is a skin condition characterized by red to blue papules on the scrotum or vulva. * ''Solitary angiokeratoma'' is a small, bluish-black, warty

that occurs predominantly on the lower extremities. * ''Verrucous vascular malformation'' (also known as "Angiokeratoma circumscriptum naeviforme") is a malformation of

dermal The dermis or corium is a layer of skin between the epidermis (with which it makes up the cutis) and subcutaneous tissues, that primarily consists of dense irregular connective tissue and cushions the body from stress and strain. It is divided ...

and subcutaneous

and

vein Veins are blood vessels in humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenat ...

s, a congenital

vascular malformation A vascular malformation is a blood vessel or lymph vessel abnormality. Vascular malformations are one of the classifications of vascular anomalies, the other grouping is vascular tumors. They may cause aesthetic problems as they have a growth cyc ...

, which, over time, a verrucous component appears.

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.

References

External links

{{Vascular tumors Dermal and subcutaneous growths