Ann Dermatol.  1994 Jan;6(1):63-68. 10.5021/ad.1994.6.1.63.

A Case of Neonatal Purpura Fulminans Due to Homozygous Protein C Deficiency

• Choi SJ
• Oh CK
• Moon DC
• Kwon KS
• Chung TA

• KMID: 2361483
• DOI: http://doi.org/10.5021/ad.1994.6.1.63

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.

Keyword

Homozygous Protein C Deficiency; Purpura Fulminans

MeSH Terms

Dermis
Hemorrhage
Humans
Infant, Newborn
Inflammation
Necrosis
Parents
Protein C Deficiency*
Protein C*
Purpura Fulminans*
Purpura*
Thrombosis
Protein C