Angiokeratoma of Mibelli in Child
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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. Females between the ages of 10 and 15 years are most commonly affected. Patients frequently complain of lesions bleeding easily with mild trauma.
There may be an association with 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.
There may be an association with 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
I78.8 – Other diseases of capillaries
SNOMEDCT:
62727008 – Angiokeratoma of Mibelli
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Last Reviewed:07/04/2022
Last Updated:07/05/2022
Last Updated:07/05/2022