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March 2005

Mali Acroangiodermatitis in Homozygous Activated Protein C Resistance

Arch Dermatol. 2005;141(3):396-397. doi:10.1001/archderm.141.3.396

We describe 2 patients whose dermatologic signs of Mali acroangiodermatitis led to the rare diagnosis of homozygous activated protein C resistance (APCR). Since its discovery in 1993, APCR has been found to be the most frequent hereditary coagulation defect. Persons with a heterozygous mutation have an increased risk of thrombosis (up to 10-fold), and the risk is up to 80-fold in persons with homozygous mutations; when thrombosis occurs, it is at a markedly younger age. Three percent of the population is heterozygous for factor V Leiden, and a very low percentage (0.02%) is homozygous.1

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