1 Department of Pediatrics, Columbia University College of Physicians and Surgeons, the Babies Hospital, and the Edward Daniels Faulkner Arthritis Clinic of the Presbyterian Hospital New York, N.Y.
A 16-year-old girl with the clinical syndrome of periodic peritonitis since early infancy was found to have elevated serum levels of unconjugated etiocholanolone during an acute attack. Biopsy of her peritoneum at that time revealed peritonitis and an abundance of mast cells. The recently described entity of Etiocholanolone Fever was briefly reviewed.