A Case of Neonatal Purpura Fulminans Due to Homozygous Protein C Deficiency.
10.5021/ad.1994.6.1.63
- Author:
Suk Joo CHOI
;
Chang Keun OH
;
Doo Chan MOON
;
Kyung Sool KWON
;
Tae Ahn CHUNG
- Publication Type:Case Report
- Keywords:
Homozygous Protein C Deficiency;
Purpura Fulminans
- MeSH:
Dermis;
Hemorrhage;
Humans;
Infant, Newborn;
Inflammation;
Necrosis;
Parents;
Protein C Deficiency*;
Protein C*;
Purpura Fulminans*;
Purpura*;
Thrombosis
- From:Annals of Dermatology
1994;6(1):63-68
- CountryRepublic of Korea
- Language:English
-
Abstract:
Homozygous Protein C deficiency is a rare genetic disease with catastrophic and fatal purpura fulminans like or thrombotic complication occurring during the neonatal period. Purpura fulminans is characterized by microvascular thrombosis in the dermis followed by perivascular hemorrhage, necrosis, and minimal inflammation. Laboratory findings are consistent with disseminated intravascular coagulopathy: We report a case of purpura fulminans in a neonate with the findings of disseminated intravascular coagulopathy and an undetectable level of protein C activity, whose parents proved to be heterozygous protein C deficiency.