A case of necrotizing fasciitis in association with hyperimmunoglobulin E (HIE) syndrome is reported. The patient was a 17-year-old Japanese boy with a clinical history of recurrent skin and pulmonary infections and eczematoid dermatitis, markedly elevated serum levels of IgE, and coarse facies. He had a gangrenous swelling on the lower abdominal wall, and his general condition was poor with high fever. The involved site was accompanied by subcutaneous gas; the culture of the pus of the lesion grew anaerobes without mixed growth of Staphylococcus aureus. Exhaustive debridement of necrotic fascia, which extended much farther than the gangrenous area, and administration of antibiotics had a curative effect on the gangrenous soft tissue infection. To the best of the authors' knowledge, this is the first published case of necrotizing fasciitis in association with HIE syndrome.
A newborn Japanese girl with Kabuki syndrome had neonatal persistent hyperinsulinemic hypoglycemia, which seemed to be a rare complication of Kabuki syndrome. On sequence analysis she was found to have a novel heterozygous KMT2D mutation. Diazoxide therapy was effective for the hypoglycemia. Hypoglycemia should be considered when Kabuki syndrome patients have convulsion or other non-specific symptoms. Diazoxide may help to improve hypoglycemia in patients with Kabuki syndrome complicated with hyperinsulinemic hypoglycemia.
A 5-day-old newborn baby presented with skin eruption, oral vesicles, and fever. His mother developed skin eruption at the same time, and his four-year-old sister was diagnosed with hand-foot-mouth disease 1 week before his delivery. Enterovirus 71 was isolated from cerebrospinal fluid that showed mild pleocytosis. This rare case of virology documented perinatal enterovirus 71 infection recovered without sequelae.
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