Recurrent Vasculopathic Skin Lesions Associated with Homozygous Protein C Deficiency
Article first published online: 2 FEB 2007
Volume 24, Issue 1, pages 57–60, January/February 2007
How to Cite
Agras, P. I., Ozdemir, H., Baskin, E. and Ozbek, N. (2007), Recurrent Vasculopathic Skin Lesions Associated with Homozygous Protein C Deficiency. Pediatric Dermatology, 24: 57–60. doi: 10.1111/j.1525-1470.2007.00335.x
- Issue published online: 2 FEB 2007
- Article first published online: 2 FEB 2007
Abstract: 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.