All issues > Volume 35(5); 1992

Case Report

J Korean Pediatr Soc. 1992;35(5):696-703. Published online May 15, 1992.

Neonatal Purpura Fulminans due to Homozygous Protein C Deficiency

Jae-Hong JH Park¹, Sung-Sub SS Sim¹, Soo-Young SY Kim¹, Hee-Joo HJ Jeon¹, Chan-Yung CY Kim¹, Hong Keun HK Oh², Jin-Yeong JY Jeong²

¹Department of Pediatrics, College of Medicine, Pusan National University, Pusan, Korea
²Department of Pediatrics, Asan Medical Center Haesung Hospital, Ulsan, Korea

Abstract

Homozygous protein C deficiency is a rare herediatary disorder of blood coagulation resulting in microvascular and venous thromboses, usually purpura fulminans, starting shortly after birth. Protein C serves to inactivate the active forms of factor V and VIII, and increase the rate of fibrin degradation. 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 due to homozygous protein C deficiency, which first apperared at age 14 hours with sudden onset of large ecchymoses on both flanks, buttocks, and thighs, and rapidly progressed. We confirmed the case by protein C study for patient and her family members, and report with the brief review of the literature.

Keywords :Homozygous protein C deficiency, Purpura fulminans