Angiokeratoma of Mibelli in Adult
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.
I78.8 – Other diseases of capillaries
62727008 – Angiokeratoma of Mibelli
Differential Diagnosis & Pitfalls