Excerpt
At 1½ months of age the patient was hospitalized for omphalitis. After that hospitalization the child had been treated three times for a chronic draining otitis media. At a follow-up visit he was febrile and irritable and was admitted for further evaluation.
On admission the temperature was 38.5°C, heart rate 140/min, blood pressure 106/60 mm Hg and respiratory rate 40/min. He was a well-developed, irritable child in no acute distress. The physical examination was remarkable for oral thrush, coarse breath sounds bilaterally and skin ulceration lateral to a 4-cm umbilical hernia. Initial laboratory evaluation revealed a white blood cell count of 65 500/mm3 with 68% segmented neutrophils, 4% band forms, 14% lymphocytes, platelets 542 000/mm3 and a hematocrit of 30.9%. Urinalysis and cerebrospinal fluid analysis were normal. Chest radiographs revealed bilateral perihilar infiltrates; abdominal films were unremarkable. The child was treated empirically with ampicillin and cefotaxime. During the ensuing week the child had continued fevers to 39.4°C. Blood, urine and cerebrospinal fluid cultures were sterile. Further laboratory evaluation revealed a negative human immunodeficiency virus antibody test, normal serum complement concentrations and normal serum immunoglobulins. T cell numbers and function were normal. A nitroblue tetrazolium test was negative. The child underwent bronchoscopy which failed to reveal any cause for the fevers.
During the second week of hospitalization the patient developed multiple pustules over the lumbar spine and buttocks which rapidly progressed to deep necrotic ulcerations. Culture of these ulcerations revealed Pseudomonas aeruginosa. Empiric antibiotics were changed to vancomycin, clindamycin and ceftazidime. A repeat complete blood cell count at that time revealed a white blood cell count of 72 800/mm3, a platelet count of 287 000/mm3 and a hematocrit of 17.7%. The necrotic wounds were surgically debrided and patient's treatment was continued with broad spectrum antibiotics and amphotericin B. Because of continuing respiratory distress and hemodynamic instability, the child required mechanical ventilation and inotropic support. A diagnostic procedure was performed at this time. Despite treatment the skin lesions progressed.
For denouement see p. 841.