This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Candece L. Gladson, MD; Paula Groncy, MD; John H. Griffin, PhD

Arch Dermatol. 1987;123(12):1701a-1706a.

Abstract
• Protein C (PC), a 62000-molecular weight vitamin K-dependent serine protease zymogen, is a natural anticoagulant that occurs in plasma at 4 mg/L. Activated PC inactivates clotting factors V and VIII and is also profibrinolytic. Activated PC is enhanced in its anticoagulant activity by protein S (PS), another vitamin K-dependent protein. Protein S is found in platelets and endothelial cells as well as in plasma. Inherited PC deficiency and PS deficiency have been associated with venous thrombosis. Both heterozygous PC and PS deficiency appear to be inherited in an autosomal dominant manner in some families. Homozygous PC deficiency presents as neonatal purpura fulminans and results in massive venous thrombosis of the skin and other organs within the first few days of life. Symptomatic heterozygous PC deficiency and PS deficiency have been treated with oral anticoagulants, successfully minimizing recurrence of thrombosis. Coumarin-induced skin necrosis, a rare complication of oral anticoagulant therapy usually seen within three to five days of initiation of therapy, has also been associated with heterozygous PC deficiency. The short half-life of PC (six to eight hours) compared with most of the vitamin K-dependent clotting factors (>30 hours) is the probable reason for this paradoxical response to oral anticoagulants in some PC-deficient patients, since a transient imbalance of procoagulant and anticoagulant factors may exist during initiation of oral anticoagulant therapy. Acquired deficiency of the PC pathway occurs in disseminated intravascular coagulation and possibly other diseases such as those associated with a lupus anticoagulant.

(Arch Dermatol 1987;123:1701a-1706a)

Author Affiliations
From the Department of Immunology, Scripps Clinic and Research Foundation, La Jolla, Calif (Drs Gladson and Griffin), and the Department of Pediatrics, Memorial Hospital Medical Center, Long Beach, Calif (Dr Groncy).

Footnotes
Accepted for publication June 19, 1987.

Read before the 36th Annual Symposium on the Biology of Skin ("Molecular Basis of Nutritional Dermatoses"), Salishan Lodge, Gleneden Beach, Ore, Oct 21, 1986.

Reprints not available.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Antithrombotic Therapy in Children*: The Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy
Monagle et al.
Chest 2004;126:645S-687S.
ABSTRACT | FULL TEXT  

Antithrombotic Therapy in Children
Monagle et al.
Chest 2001;119 :344S-370S.
FULL TEXT  

Erythematous, Hemorrhagic, and Necrotic Plaques in an Elderly Man
Sharafuddin et al.
Arch Dermatol 1992;128:105-106.
ABSTRACT  

Prothrombotic phenotype of protein Z deficiency
Yin et al.
Proc. Natl. Acad. Sci. USA 2000;97:6734-6738.
ABSTRACT | FULL TEXT