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- Title
Recurrent Vasculopathic Skin Lesions Associated with Homozygous Protein C Deficiency.
- Authors
Agras, Pinar Isik; Ozdemir, Handan; Baskin, Esra; Ozbek, Namik
- 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.
- Subjects
DEFICIENCY diseases; PROTEIN C deficiency; RARE diseases; VASCULAR diseases; BOYS; PEDIATRIC dermatology; PURPURA (Pathology)
- Publication
Pediatric Dermatology, 2007, Vol 24, Issue 1, p57
- ISSN
0736-8046
- Publication type
Article
- DOI
10.1111/j.1525-1470.2007.00335.x