Korean J Dermatol.  2002 Jan;40(1):38-43.

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

• Jo JH
• Oh CK
• Kim MB
• Jang HS
• Kwon KS

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.

Keyword

Purpura fulminans; Homozygous protein C deficiency; Fresh frozen plasma

MeSH Terms

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
Protein C
Warfarin