Mseleni joint disease (MJD) and Handigodu joint disease (HJD) are familial skeletal disorders that affect several hundred persons in northern Zululand, South Africa and in the Shimoga district of southern India, respectively. Severe precocious, progressive degenerative osteoarthropathy, which occurs in both conditions, causes marked physical handicap by adulthood. The clinical and radiological manifestations of MJD and HJD are very similar and it is possible that they represent the same entity. HJD appears to be inherited as an autosomal dominant trait, while MJD clusters in families without a definite Mendelian pattern. It is possible that an environmental factor is active in the pathogenesis of both disorders and comparative investigations would be fruitful. The predominance of severe degenerative osteoarthropathy in both disorders may have important implications for the elucidation of the pathogenesis of the common conventional forms of osteoarthropathy.