Abstract
A boy with hyperimmunoglobulin E syndrome (HIE syndrome), who was successfully treated with long-term sulfamethoxazole-trimethoprim (SMX-TMP) is reported. He had been suffering from recurrent pruritic dermatitis soon after birth and had a significant high level of serum immunoglobulin E. Although an initiation of SMX-TMP therapy resulted in resolution of his clinical manifestations, cessation of the treatment exacerbated the symptoms. Chemoprophylaxis of other oral antibiotics, which were suitable for Staphylococcus aureus isolated from lesions of the patient were unsuccessful. Another trial of low-dose SMX-TMP therapy resulted in gradual subsidence of the clinical manifestations. From these observations, efficacy of SMX-TMP therapy to prevent bacterial infection in the patient is clinically apparent. Although precise mechanism of the therapy remains speculative, long-term SMX-TMP therapy might be of benefit and low clinical toxicity in HIE syndrome.