Article

Hand-Schüller-Christian Disease with Unusual Roentgenographic Findings Occurring in an Adult

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Abstract

THE usual roentgenographic manifestions of Hand-Schüller-Christian disease consist of a few radiolucent defects of moderate-to-large size in the calvaria and sometimes in the peripheral bones. These lesions most often occur in children, rarely in adults. Our purpose is to report a case of Hand-Schüller-Christian disease in an adult with unusually extensive involvement of the long bones in addition to the common roentgenographic findings.

Case Report

A 40-year-old white man was first examined at the Cleveland Clinic in March, 1954, because of excessive thirst (consuming two gallons of water daily), frequency of urination, and loss of libido. On physical examination the only abnormal finding was small testes. Roentgenograms of the skull were normal. The chest roentgenogram showed evidence of a finely nodular infiltrate, particularly in the upper lobes, suggestive of sarcoidosis; however, the Nickerson-Kveim test was negative. The clinical impression was that of pituitary insufficiency with diabetes insipidus.

Two years later, in 1956, he was re-examined because of multiple symptoms, including the recurrence of those of pituitary insufficiency, generalized weakness, and pain in the ankles. Roentgen examination at that time revealed no change in the appearance of the chest. Roentgenograms of the skull again demonstrated evidence of a normal sella turcica, but there were two radiolucent areas in the parietal region. Roentgenograms of the right ankle gave evidence of a pathologic fracture of the talus with collapse, and obvious bony defects about the ankle (Fig. 1). Bone survey studies evidenced diffuse involvement of the skeleton, with the exception of the spine, . . .


 

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