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Acute hemorrhagic edema of infancy.

Patient Description An 11 month old boy was admitted because of fever, edema and rash. His history and that of his family were unremarkable. He had diarrhea for 3 days and a rash appeared on his thighs on the day before admission. Examination revealed an ecchymotic rash over the face, auricles, arms, hands, gluteal area, legs and feet. The lesions, which were tender, initially resembled insect bites but gradually became larger, concentric and purpuric [Figure]. The child also had a fever of 39.5C and non-pitting edema of the face and legs. Other vital signs and the rest of the physical examination were unremarkable. Blood laboratory tests showed a white blood cell count of 18,000/mm, hemoglobin 12 g/dl, platelet count 532,000/mm and erythrocyte sedimentation rate 35 mm/ hour. Urinalysis showed 4±5 red blood cells/high power field. Blood chemistry (SMA-18), coagulation studies and sepsis workup, including lumbar puncture, were all within normal limits. Serum immunoglobulin and complement levels were normal. Blood urine and stool cultures were negative. Skin biopsy showed leukocytoclastic vasculitis with nuclear dust mixed with leukocytic infiltrate in the vessel walls. Direct immunofluorescence revealed a mild perivascular deposit of C3 but no deposits of immunoglobulins. A diagnosis of acute hemorrhagic edema of infancy was made. During hospitalization, bloody diarrhea was noted. Intravenous ceftriaxone (100 mg/kg/day) was administered for 3 days until the fever gradually normalized, and the blood disappeared from the stools. Five days after admission the skin lesions began to clear and the edema subsided. New purpuric lesions recurred during the following 3 weeks, but afterwards the skin cleared and there were no further recurrences for 12 months of follow-up.