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Angiokeratoma of Mibelli in Adult
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Angiokeratoma of Mibelli in Adult

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Contributors: Susan Burgin MD, Chris G. Adigun MD, David Dasher MD, Jeffrey D. Bernhard MD
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Synopsis

Angiokeratoma of Mibelli (AKM), also known as the angiokeratoma of Mibelli of adolescence, is a rare, acquired, localized form of angiokeratoma that typically occurs on acral sites. Young females between the ages of 10 and 15 are most commonly affected. Patients frequently complain of lesions bleeding easily with mild trauma.

Many patients have a history of episodes of perniosis and acrocyanosis. It is theorized that these cyanotic attacks have deleterious effects on vessel walls that lead to subsequent development of vascular ectasia. This etiological theory, though, has been debated. Associated systemic vascular involvement has not been established. However, necrosis of the fingertips in AKM has been described.

Although AKM is considered to be an acquired disease, genetic predisposition is likely, and familial studies have revealed higher incidences among siblings than expected.

Codes

ICD10CM:
I78.8 – Other diseases of capillaries

SNOMEDCT:
62727008 – Angiokeratoma of Mibelli

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Last Updated: 10/27/2017
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Angiokeratoma of Mibelli in Adult
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Angiokeratoma of Mibelli : Acral, Hemorrhagic papule
Clinical image of Angiokeratoma of Mibelli
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