Purpura fulminans. Medical, surgical, and rehabilitative considerations.
Purpura fulminans is a rare catastrophic disease of childhood that begins as a seemingly benign infectious process and subsequently progresses to high fever, purpuric ecchymosis of the skin, hypotension, disseminated intravascular coagulation, and gangrene. Initial treatment consists of resuscitation with Ringer's lactate, correction of clotting parameters with heparin, avoidance of adrenergic agents, and monitoring of limbs for compartment syndromes. Of 12 patients reported here, three had fasciotomies to limit the ischemic changes, but eventually 24 amputations were performed on the group as a whole. Delayed debridement of up to three weeks was the rule in order to allow ischemic tissue to fully demarcate. Split-thickness skin grafts were used to preserve as much length as possible, particularly where open growth plates were involved. Regardless of how hopeless the situation may initially appear, with prompt recognition and institution of appropriate care the prognosis for a productive life is good.
D000293:Adolescent; D000671:Amputation, Surgical; D002648:Child; D002675:Child, Preschool; D003161:Compartment Syndromes / Q000209:etiology / Q000601:surgery; D004211:Disseminated Intravascular Coagulation / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D007511:Ischemia / Q000209:etiology*; D007866:Leg / Q000098:blood supply* / Q000601:surgery; D008297:Male; D011693:Purpura / Q000150:complications* / Q000628:therapy; D011788:Quality of Life