A Case of Neonatal Purpura Fulminans Due to a Homozygous Protein C Deficiency.
- Author:
Ju Hyun JO
1
;
Chang Keun OH
;
Moon Bum KIM
;
Ho Sun JANG
;
Kyung Sool KWON
Author Information
1. Department of Dermatology, College of Medicine, Pusan National University Pusan, Korea. ckoh@pusan.ac.kr
- Publication Type:Case Report
- Keywords:
Purpura fulminans;
Homozygous protein C deficiency;
Fresh frozen plasma
- MeSH:
Blood Coagulation Disorders, Inherited;
Capillaries;
Debridement;
Disseminated Intravascular Coagulation;
Early Diagnosis;
Ecchymosis;
Humans;
Infant;
Infant, Newborn;
Necrosis;
Parents;
Plasma;
Protein C Deficiency*;
Protein C*;
Purpura Fulminans*;
Purpura*;
Recurrence;
Skin;
Thrombosis;
Transplants;
Venules;
Warfarin
- From:Korean Journal of Dermatology
2002;40(1):38-43
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Homozygous protein C deficiency is a rare hereditary coagulation disorder that occurs most often in childhood and is characterized by widespread thrombosis of capillaries and venules, abrupt onset of ecchymoses and necrosis. The hematological feature corresponds with disseminated intravascular coagulation. Protein C is a natural anticoagulant and also has important anti-inflammatory activity. For confirmation of homozygous protein C deficiency, the infant should have undetectable protein C activity and both parents should be heterozygous for protein C deficiency. We experienced a case of purpura fulminans in the newborn infant in whom we identifed homozygosity for familial protein C deficiency. Fresh frozen plasma for replacement of protein C, early debridement and full-thickness skin graft induced a remission. Administration of warfarin was used to prevent recurrence of attacks. This report emphasizes the need for early diagnosis and adequate replacement therapy in patient with purpura fulminans.
