MEMBRANOUS LIPODYSTROPHY OF BONE

  • 1 January 1978
    • journal article
    • research article
    • Vol. 102  (1) , 22-27
Abstract
A 23 yr old man with a fracture of the right lateral malleolus had osteolytic lesions of the lower end of both fibulas and of the right talus. He was mentally defective from birth, with convulsions from the age of 4 yr and a limp since the age of 15. Biopsy tissue from the right fibula that was removed 1 yr after the fracture consisted of marrow adipose tissue that was transformed by the development of thick, convoluted PAS [periodic acid Schiff] positive membranes around the cells. EM indicated that each membrane had a complex microvillous substructure. At the time of biopsy, lytic lesions were found in both femoral medial condyles and, 1 yr later, in the distal shaft of the left radius. Membranous lipodystrophy is a rare generalized skeletal disease that was previously reported in Finland and Japan, and is usually followed by progressive presenile dementia.

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