Membranous lipodystrophy apparently is a new disease, first reported and
named by Nasu and associates 31 in 1971. We have collected six cases of
this disease. The initial manifestation was articular pain at adolescence.
Symmetrical changes in the bones of the extremities then appeared and the
disease seemed to progress slowly with age. The younger patients exhibited
only skeletal pain or fractures, while the older patients had some
neuropsychiatric changes. Three of the patients died in middle age. The
cystic bone lesions contained a yellow, lipid-like substance which
histologically showed a characteristic membranocystic appearance. Electron
microscopic study showed unique features, but biochemical analysis of the
substance did not reveal a definable abnormality.