Hepatolenticular Degeneration Associated with Acanthosis Nigricans

Hepatolenticular degeneration is a relatively rare disease. Its clinical and laboratory features are well known but poorly understood. It was thought desirable to record the findings on this patient in order to report the association of this disease with acanthosis nigricans and to describe this patient's course following treatment. REPORT OF CASE The patient, a 15-year-old white girl, was admitted to Firmin Desloge Hospital on March 29, 1956, complaining of swelling of her abdomen. She had lived on a Missouri farm and had been in good health until approximately one month prior to admission, when she consulted her family physician because of delay in menarche. At this time she received one injection of chorionic gonadotropin, and shortly she thereafter noticed swelling of her feet and ankles. During the four weeks preceding entry there was a rapid progression of the edema, with a 40 lb. weight gain and development of ascites.