成人に発症したＨａｎｄ‐Ｓｃｈｕｅｌｌｅｒ‐Ｃｈｒｉｓｔｉａｎ病の１例

Abstract

Hand-Schuller-Christian Disease is usually diagnosed in children and young adults. An unusual case of Hand-schuller-Christian disease with severe pulmonary involvement in a middle aged man was reported. A 48 years old man complaining of gait disturbance came to our clinic in June 1988. He had noticed polydipsia and polyuria since 1980, but worked as a taxi driver. He had noticed the onset of pain in both knees in Oct. 1986 and subsequently became incapaciated and was able to move only with assistance. Roentgenograms showed symmetrical sclerosis involving metaphysis and diaphysis of the proximal ulna, proximal humerus and distal femur. There were osteolytic lesion in the sclerotic area in the proximal tibia, femur and acetabular region of the left pelvis and pathologic supracondylar fracture of the both femurs.He voiced 10 liters of urine with a specific gravity less than 1.002. Open biopsy of the tibia revealed numerous foamy histiocytes in a fibrotic bed.The patient is under the treatment with Prednisolone, Desmo pressin and training with cruch gait.