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 Pathol ...

. ''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, brain, and skin. Fabry disease is one of a group of conditions known as lysosomal storage dis ...

, 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 ...

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

, 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, brain, and skin. Fabry disease is one of a group of conditions known as lysosomal storage dis ...

, 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, a doctor may recommend excision and grafting.

Pathophysiology

Histology

Angiokeratomas characteristically have large dilated

blood vessel Blood vessels are the tubular structures of a circulatory system that transport blood throughout many Animal, animals’ bodies. Blood vessels transport blood cells, nutrients, and oxygen to most of the Tissue (biology), tissues of a Body (bi ...

s in the superficial

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

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 lesion, skin. It may have a rounded, pointed or flat top, and may have a umbilication, dip. It can appear with a Peduncle (anatomy), stalk, be thread-like or look warty. It can be soft or firm a ...

s made by dilatated

capillaries A capillary is a small blood vessel, from 5 to 10 micrometres in diameter, and is part of the microcirculation system. Capillaries are microvessels and the smallest blood vessels in the body. They are composed of only the tunica intima (the in ...

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

File:SkinTumors-PA090939.JPG, Angiokeratoma (Mibelli type)

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 medi ...

Vittorio Mibelli (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) 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 i ...

and subcutaneous

and

vein Veins () are blood vessels in the circulatory system of 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 those of the pulmonary and feta ...

s, a congenital

vascular malformation A vascular malformation is a type of vascular anomaly. They may cause aesthetic problems as they have a growth cycle, and can continue to grow throughout life. Vascular malformations of the brain include those involving capillaries, and those in ...

, which, over time, a verrucous component appears.

Treatment

Different treatment options exist including cryotherapy, laser therapy, and excision.

References

External links

{{Vascular tumors Dermal and subcutaneous growths