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Purpura fulminans with Low Protein C in Meningococcal and Pneumococcal Septicemia
Ist Teil von
28. Hämophilie-Symposion Hamburg 1997, p.380-390
Ort / Verlag
Berlin, Heidelberg: Springer Berlin Heidelberg
Link zum Volltext
Beschreibungen/Notizen
Purpura fulminans is a cutaneous lesion with fibrination of the microvasculature, erythrocyte extravasation and granulocytic reaction. It appears in different infectious disease states such as meningococcal or pneumococcal septicemia or secondary to preceding (viral or bacterial) infections or for unknown causes [1]. The purpuric lesions usually progress to necrosis and even extremity gangrene may develop. Other organs may be involved. Powars et al. [2] were the first to suggest that low levels of protein C may contribute to the pathogenesis of purpura fulminans in meningococcal septicemia. Purpura fulminans also occurs in homozygous inherited deficiencies of the protein C anticoagulant pathway in the perinatal period [3, 20, 21] and as a complication of coumarin therapy [4]. The common denominator in these clinical settings is thought to be a low or missing protein C activity.