Homozygous protein C deficiency with late onset and recurrent coumarin-induced skin necrosis

The Lancet ◽  
1992 ◽  
Vol 339 (8795) ◽  
pp. 743-744 ◽  
Author(s):  
J. Conard ◽  
M.H. Horellou ◽  
P. Van Dreden ◽  
M. Samama ◽  
P.H. Reitsma ◽  
...  
Keyword(s):  
Skin Necrosis ◽  
Protein C ◽  
Late Onset ◽  
Protein C Deficiency

Problems of Oral Anticoagulation in an Adult with Homozygous Protein C Deficiency and Late Onset of Thrombosis

1993 ◽  
Vol 69 (04) ◽  
pp. 311-315 ◽  
Author(s):  
P Pescatore ◽  
H M Horellou ◽  
J Conard ◽  
M Piffoux ◽  
P Van Dreden ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Oral Anticoagulation ◽  
Skin Necrosis ◽  
Protein C ◽  
Low Molecular Weight Heparin ◽  
Late Onset ◽  
Low Molecular Weight ◽  
Activity Levels ◽  
Protein C Deficiency ◽  
New Therapeutic Approaches

SummaryWe describe a 57-year-old woman with homozygous protein C deficiency and mild thrombotic manifestations consisting of three spontaneous distal deep vein thromboses occurring after the age of 45. Previous surgery and pregnancies had been uneventful. Low but detectable protein C antigen and activity levels (both 20%) were discovered on the occasion of skin necrosis induced by oral anticoagulation. This therapy was interrupted because of skin necrosis and several episodes of disseminated intravascular coagulation (DIC) at the initiation of treatment despite a cautious protocol. No recurrent thromboembolic event has occurred in our patient using prophylactic doses of low molecular weight heparin for 24 months. New therapeutic approaches might be the administration of low molecular weight heparin or oral anticoagulation associated with protein C replacement in the induction period. This case reflects the variability of expression of protein C deficiency as well as the potential hazards of antivitamin K anticoagulation in this disorder.

scholarly journals Late-Onset Homozygous Protein C Deficiency Manifesting Cerebral Infarction as the First Symptom at Age 27.

1992 ◽  
Vol 31 (7) ◽  
pp. 922-925 ◽  
Author(s):  
Katsumi DEGUCHI ◽  
Tetsuya TSUKADA ◽  
Eiichi IWASAKI ◽  
Hideo WADA ◽  
Seiko MURASHIMA ◽  
...  
Keyword(s):  
Cerebral Infarction ◽  
Protein C ◽  
Late Onset ◽  
Protein C Deficiency

Childhood-onset skin necrosis resulting from protein C deficiency

2006 ◽  
Vol 0 (0) ◽  
pp. 070209222700073-???
Author(s):  
PR Criado ◽  
IM Bernardelli ◽  
EA Rivitti ◽  
MN Sotto ◽  
MAC Vilella ◽  
...  
Keyword(s):  
Skin Necrosis ◽  
Protein C ◽  
Childhood Onset ◽  
Protein C Deficiency

Late-onset homozygous protein C deficiency

The Lancet ◽  
1991 ◽  
Vol 338 (8766) ◽  
pp. 575-576 ◽  
Author(s):  
C.B Grundy ◽  
E Melissari ◽  
V Lindo ◽  
M.F Scully ◽  
V.V Kakkar ◽  
...  
Keyword(s):  
Protein C ◽  
Late Onset ◽  
Protein C Deficiency

scholarly journals NECROSE CUTANEE REVELANT UN DEFICIT CONGENITAL EN PROTEINE C : A PROPOS DE 3 CAS

2021 ◽  
Vol 9 (03) ◽  
pp. 80-83
Author(s):  
S. Halouani ◽  
◽  
W. Kojmane ◽  
F. Hmami ◽  
S. Atmani ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Replacement Therapy ◽  
Disseminated Intravascular Coagulation ◽  
Skin Necrosis ◽  
Protein C ◽  
Autosomal Recessive ◽  
Autosomal Recessive Disorder ◽  
Protein C Deficiency ◽  
Neonatal Skin ◽  
Protein C Activity

Neonatal skin necrosis in the context of a congenital homozygous protein C deficiency is a rare inherited autosomal recessive disorder, it is characterized by rapidly extensive necrotic patches occurring a few hours after birth in a newborn who doesnt present any hemodynamic disorder. The diagnosis is based on the assay of protein C activity which is collapsed or even undetectable. Early diagnosis and replacement therapy are the mainstays of management before the onset of disseminated intravascular coagulation. We report three cases of newborns presenting with DIC in the context of protein C deficiency and the course of which was fatal.

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