[Histiocytosis X. Follow-up for over 20 years in a patient with Hand-Schüller-Christian disease].

Since 1963 we have observed a patient with Hand-Schüller-Christian disease with involvement of the skeleton, skin and hypophysis/hypothalamus with diabetes insipidus. The diabetes insipidus was successfully treated initially with chlorpropamid and later with carbamazepin. Alterations of the lung parenchyma were seen radiologically in 1966, and two years later the patient complained of exertional dyspnea. The lung disease was progressive with the deterioration of lung function despite corticosteroid and D-penicillamine treatment. The patient now has marked dyspnea even at rest. The osteolytic lesions have not changed in recent years.