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WaterhouseFriderichsen Syndrome
Two hours after the onset of fever and malaise, a 15-year-old boy began to have tachycardia and tachypnea and became unconscious. He was taken to the intensive care unit, where he was found to be in septic shock (pulse, 180; blood pressure, 70/45 mm Hg; temperature, 40.2C; and partial-thromboplastin time, 90 seconds). He had bullous lesions about the elbow and confluent petechial and purpuric lesions on his face, arms, and legs (Panel A). His hands and feet were cool and cyanotic. He had no signs of meningeal irritation. He was intubated, placed on cardiopulmonary support, and given large doses of catecholamines. A single dose of dexamethasone (3 mg per kilogram of body weight) was given immediately, and treatment with penicillin G (16 million IU per square meter of body-surface area) was started. Meanwhile, the petechiae spread rapidly, and four hours later peripheral pulses could not be detected in the arms or legs. Therapy with fresh-frozen plasma and heparin was also begun. The working diagnosis of meningococcemia was later confirmed by the finding of serogroup B Neisseria meningitidis in blood samples and in samples obtained from the affected skin. Although the treatment led to resolution of the sepsis syndrome, local intravascular lysis with streptokinase failed to prevent severe ischemia-induced damage to the patients hands (Panel B) and feet (Panel C). Seven digits and both forefeet had to be amputated. After reconstructive surgery with free flaps, the patient was able to walk with orthopedic shoes and could use his remaining fingers in a pincer-like grip. THOMAS SCHOELLER, M.D. ERICH SCHMUTZHARD, M.D.
Copyright 2001 Massachusetts Medical Society.

Leopold-Franzens University Hospital A-6020 Innsbruck, Austria

1372 N Engl J Med, Vol. 344, No. 18 May 3, 2001 www.nejm.org

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