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September 1988

Purpura Fulminans and Transient Protein C and S Deficiency

Author Affiliations

Department of Dermatology Baylor College of Medicine Houston, TX 77030

Arch Dermatol. 1988;124(9):1442-1443. doi:10.1001/archderm.1988.01670090084024

To the Editor.—  Purpura fulminans is characterized by purpura, disseminated intravascular coagulation, hypotension, and fever. Bacterial or viral infections usually precede its development. The exact pathogenesis is unknown, but a generalized Shwartzmann reaction has been accepted as the most likely cause.1When recently presented with a patient in whom purpura fulminans developed, we were impressed by the morphologic similarity of the skin lesions to those of coumarin necrosis. Because coumarin necrosis has been associated with heterozygous protein C deficiency,2 we investigated this possibility in our patient. On review of the literature, purpura fulminans has been described in neonatal homozygous protein C deficiency and acquired protein C and S deficiencies,3,4 but heterozygous protein C deficiency in adults with purpura fulminans has not been documented. Interestingly, the findings in our patient were those of transient protein C and S deficiencies.

Report of a Case.—  An 18-year-old man was admitted