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Recurrent Vasculopathic Skin Lesions Associated with Homozygous Protein C Deficiency
Author(s) -
Agras Pinar Isik,
Ozdemir Handan,
Baskin Esra,
Ozbek Namik
Publication year - 2007
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2007.00335.x
Subject(s) - medicine , purpura fulminans , protein c deficiency , protein s deficiency , pathology , purpura (gastropod) , dermatology , protein c , protein s , thrombosis , venous thrombosis , ecology , biology
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.