Hyperimmunoglobulin E syndrome associated with nephrotic syndrome

A 21-year-old man was admitted to Kure National Hospital with nephrotic syn drome in September 1996. He had suffered from an intractable pruritic skin rash and recurrent subcutaneous abscesses caused by the hyperimmunoglobulin E syndrome since the age of 18 months, Renal biopsy gave a diagnosis of me mbranoproliferative glomerulonephritis, Steroid therapy decreased urinary p rotein loss and hypoproteinemia, and his pruritic skin rash was improved. P atients with hyperimmunoglobulin E syndrome have a defective immune respons e, especially to Staphylococcus aureus infection. Continuous antigen stimul ation may have caused this patient's renal histological damage as in immune complex glomerulonephritis.