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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

Report of Cases

Case 1

A 59-year-old woman had dark brown–purplish confluent patches and plaques as well as edema on the left medial and lateral malleolus, forefoot, and distal lower leg for 15 years (Figure). Her medical history revealed left pelvic vein thrombosis of unknown origin at the age of 30 years. She received oral anticoagulation treatment for some . . . [Full Text of this Article]

Case 2

Comment

AUTHOR INFORMATION
Susanne Scholz, MD; Sanja Schuller-Petrovic, MD, PhD; Helmut Kerl, MD, PhD