ABSTRACT

The multicentric osteolysis disorders, sometimes called vanishing bones syndromes are a group of inherited disorders of bone characterized by progressive destruction of bones and joints leading to skeletal deformities and functional impairment. Inheritance may be autosomal recessive or dominant, and there may or may not be associated nephropathy or mental retardation [1]. The disorders have been classified by the international skeletal dysplasia society into four groups (Table 112.1) [2]. They are notable for erosions in the interphalangeal joints that mimic severe juvenile rheumatoid arthritis (OMIM 166300, 259600, 259610, and 277950).