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Purpura FulminansA Cutaneous Manifestation of Severe Protein C Deficiency
Michael J. Auletta, MD;
John T. Headington, MD
Arch Dermatol. 1988;124(9):1387-1391.
Abstract
• Protein C, when activated, is a vitamin K-dependent serine protease that has anticoagulant and profibrinolytic activities. An increased risk of thrombotic disease is associated with deficiency of this enzyme. A neonate developing purpura fulminans with internal thrombotic complications and congenital absence of protein C is described. Family studies showed partial protein C deficiency in the subject's symptom-free parents and two of three siblings. Clinically and histopathologically similar lesions are also seen in two acquired conditions in which there is a thrombotic tendency: coumarin necrosis and purpura fulminans of childhood, both of which are probably the result of transient protein C deficiency. We conclude that purpura fulminans can represent a cutaneous marker of acquired or congenital protein C deficiency.
(Arch Dermatol 1988;124:1387-1391)
Author Affiliations
From the Departments of Dermatology (Dr Auletta) and Pathology (Dr Headington), University of Michigan Medical School, Ann Arbor.
Footnotes
Accepted for publication Feb 21, 1988.
Presented in part at the 47th annual meeting of the American Academy of Dermatology, San Antonio, Tex, Dec 7, 1987.
Reprint requests to Department of Pathology, University of Michigan Medical School, Medical Science I M5242/0602, 1301 Catherine, Ann Arbor, MI 48109-0602 (Dr Headington).
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