Angiokeratoma is a benign cutaneous lesion of capillaries, resulting in small marks of red to blue color and characterized by hyperkeratosis. Angiokeratoma corporis diffusum refers to Fabry's disease,[1] but this is usually considered a distinct condition.
Contents
1Signs and symptoms
1.1Complications
2Pathophysiology
2.1Histology
3Diagnosis
3.1Classification
4Treatment
5See also
6References
7External links
Signs and symptoms
Angiokeratoma of Fordyce
Presentation includes telangiectasia, acanthosis, and hyperkeratosis.[2]
Presentation can be solitary or systemic.[3]
Multiple angiokeratomas, especially on the trunk in young people, are typical for Fabry disease, 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 vessels in the superficial dermis and hyperkeratosis (overlying the dilated vessels).
Scrotal angiokeratoma; visible large dilated blood vessels and hyperkeratosis
Scrotal angiokeratoma (Fordyce type); multiple papules made by dilatated capillaries
Scrotal angiokeratoma (Mibelli type); blood vessels close to the epidermis
Angiokeratoma (Mibelli type)
Diagnosis
Due to the rarity of different types of vascular conditions, angiokeratomas may be misdiagnosed. A biopsy of the lesion can produce a more accurate diagnosis.
Classification
Angiokeratoma may be classified as:
Angiokeratoma of Mibelli (also known as "Mibelli's angiokeratoma,"[4] "Telangiectatic warts"[5]) consists of 1- to 5-mm red vascular papules, the surfaces of which become hyperkeratotic in the course of time.[5]:589 The disease is named after Italian dermatologist Vittorio Mibelli (1860-1910).[6]
Angiokeratoma of Fordyce (also known as "Angiokeratoma of the scrotum and vulva," though not to be confused with Fordyce's spots)[5] is a skin condition characterized by red to blue papules on the scrotum or vulva.
Solitary angiokeratoma is a small, bluish-black, warty papule that occurs predominantly on the lower extremities.[5]:590
Verrucous vascular malformation (also known as "Angiokeratoma circumscriptum naeviforme") is a malformation of dermal and subcutaneous capillaries and veins, a congenital vascular malformation, which, over time, a verrucous component appears.[5]:584
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.
See also
Fabry disease
List of cutaneous conditions
References
^Trickett R, Dowd H (October 2006). "Angiokeratoma of the scrotum: a case of scrotal bleeding". Emerg Med J. 23 (10): e57. doi:10.1136/emj.2006.038745. PMC 2579622. PMID 16988295.
^"angiokeratoma" at Dorland's Medical Dictionary
^Sion-Vardy N, Manor E, Puterman M, Bodner L (January 2008). "Solitary angiokeratoma of the tongue" (PDF). Med Oral Patol Oral Cir Bucal. 13 (1): E12–4. PMID 18167473.
^Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
^ abcdeJames, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
…increase in number with pregnancy. The diagnosis is clinical, based on characteristic appearance. Angiokeratomas are asymptomatic, dark-red, purple, blue, or black, smooth-surfaced, dome-shaped, benign papules …
…dermatofibroma, basal cell carcinoma, squamous cell carcinoma, seborrheic keratosis, or angioma/hemangioma/angiokeratoma. Lesions lacking features to classify them as one of the aforementioned categories are evaluated …
…The features are similar to those observed in congenital nevi of the skin. Hemangiomas and angiokeratomas show clods and/or structureless areas of red and blue colors; occasionally, lesions show white…
…(LVH), and angiokeratomas, are not typical in patients with psychiatric disease. Fibromyalgia. As above, many common manifestations of Fabry, including proteinuria, LVH, and angiokeratomas, are not common …
… Fabry disease (MIM 300644, also known as angiokeratoma corporis diffusum, ceramide trihexosidosis, or Anderson-Fabry disease) is an X-linked glycolipid storage disease . It is caused by deficient activity …
English Journal
Enzyme replacement therapy in two Japanese siblings with Fabry disease, and its effectiveness on angiokeratoma and neuropathic pain.
Furujo M, Kubo T, Kobayashi M, Ohashi T.SourceDepartment of Pediatrics, Okayama Medical Center, Okayama, Japan. Electronic address: fuhkun@okayama3.hosp.go.jp.
Enzyme replacement therapy (ERT) for Fabry disease does not show a clear benefit in angiokeratoma. We describe two Japanese siblings with Fabry disease, who were diagnosed when angiokeratomas were found on the older sibling at the age of 13years. Neither of the boys complained of pain, while both su
O'Mahony C, Franks A, Llewellyn R.SourceDepartment of Sexual Health & HIV, Countess of Chester Hospital, Chester, UK.
International journal of STD & AIDS.Int J STD AIDS.2013 Oct 8. [Epub ahead of print]
Many patients have few scattered angiokeratoma and we reassure them that this it is normal; however, if they are numerous, Fabry disease should be considered and check family history.
Angiokeratoma a is benign cutaneous lesion of capillaries, resulting in small marks of red to blue color and characterized by hyperkeratosis. "Angiokeratoma corporis diffusum" refers to Fabry's disease, but this is usually considered a distinct ...
