[Fabry disease, a disease with rheumatic aspects: radiology of soft tissue and bone changes in the hand].

Fabry's disease is a hereditary lipid storage disease with deposition of ceramides in nearly all tissues. The lipid deposition in the skin and the synovial compartments causes an enlargement of the skin, the joint capsules and the tendon sheaths, which are recognizable by low KV radiography. The bones of the hand mainly show multiple enthesopathic ossifications of a peculiar kind at the insertions of fibrous structures and few intra- and extraarticular erosions. The clinical picture often simulates an inflammatory rheumatic disease.