Purpura Fulminans: Report of a case successfully treated with heparin and hydrocortisone Review of 50 cases from the literature

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Abstract
Severe purpura fulminans (p. f.) developed in a three year old boy four days after an attack of diarrhea. Streptococci were found in his throat. He went into shock following massive bleeding into the skin. Haemostatic studies revealed moderate thrombocytopenia and profound clotting defects compatible with intravascular coagulation. Skin biopsies disclosed thrombosis of small blood vessels. He improved dramatically after treatment with heparin and hydrocortisone; his clotting factors quickly returned to normal. Following extensive skin grafting, he made a complete recovery.Fifty selected cases of p. f. have been reviewed. P. f. appears to occur only in children, usually 1–4 weeks after a benign infection, such as scarlatina or varicella. The clinical findings have been described in detail. All patients appear to have one or more episodes of intravascular coagulation resulting in secondary haemostatic defects.A concept of pathogenesis has been presented in terms of (1) preparation of the skin by mechanism as yet unknown, followed by (2) an episode of acute intravascular coagulation which deposits thrombi in small blood vessels of the prepared skin by mechanisms as yet unknown, followed by (2) an episode of acute intra‐been discussed. Therapy should be based upon heparin to stop intravascular clotting and adrenal steroids to modify possible antigen‐antibody reactions.