Recurrent vasculopathic skin lesions associated with homozygous protein C deficiency.
Symptomatic protein C deficiency is a rare condition. Vasculopathy associated with hypercoagulable state in protein C deficiency has also been reported rarely. We described a boy who was diagnosed as having homozygous protein C deficiency during the neonatal period, when he developed purpura fulminans. At 7 years of age, he developed recurrent, painful, nonscarring, purpuric skin lesions. Histopathologic skin findings were compatible with those of vasculopathy. The histopathologic characteristics of these vasculopathic lesions and the pathogenetic mechanisms of their association with protein C deficiency are discussed.
Agras
Pinar Isik
PI
Baskent University Department of Pediatric Nephrology, 6 Cadde No. 72/3, Bahcelievler, 06490 Ankara, Turkey. pinaris2001@yahoo.com
Ozdemir
Handan
H
Baskin
Esra
E
Ozbek
Namik
N
D002648:Child; D006801:Humans; D008297:Male; D016491:Peripheral Vascular Diseases / Q000150:complications* / Q000473:pathology; D020151:Protein C Deficiency / Q000150:complications*; D011693:Purpura / Q000209:etiology* / Q000473:pathology; D012008:Recurrence