A Case of Hereditary Deficiency of Protein C (Homozygous) with Wide Spread Skin Necrosis.
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概要
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We report a case of hereditary deficiency of protein C (homozygous) with wide spread skin necrosis in a two-month-old baby. He presented with large purpura on his lower half of the abdomen and scrotal skin with severe fever two days after his birth. Laboratory examinations revealed that protein C activity and antigenesity was undetectable in the patient and that both of his parents showed half of the activity. Computed tomography and magnetic resonance imaging revealed the multiple cerebral infarctions in his left cerebrum. Although the ulcers gradually decreased the size during the treatment with fresh frozen plasma and anticoagulants, he developed purpura on his ankles and scalp again by the discontinuation of the treatments.<BR>We discuss the relationship between the biochemical properties of protein C and clinical aspects of the deficiency.
- 日本皮膚科学会大阪地方会の論文
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