Angiokeratoma of Mibelli in Adult
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Synopsis
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
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Differential Diagnosis & Pitfalls
- Hemangioma
- Lobular capillary hemangioma (pyogenic granuloma)
- Angioma serpiginosum
- Glomangioma
- Lymphangioma circumscriptum
- Kaposi sarcoma
- Melanocytic nevus
- Spitz nevus
- Melanoma
- Angiokeratoma corporis diffusum (Fabry disease)
- Fucosidosis
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Last Reviewed:07/04/2022
Last Updated:07/05/2022
Last Updated:07/05/2022
Angiokeratoma of Mibelli in Adult