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September 1988

Purpura Fulminans: A Cutaneous Manifestation of Severe Protein C Deficiency

Author Affiliations

From the Departments of Dermatology (Dr Auletta) and Pathology (Dr Headington), University of Michigan Medical School, Ann Arbor.

Arch Dermatol. 1988;124(9):1387-1391. doi:10.1001/archderm.1988.01670090043009

• 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)

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