The clinical features, investigation, treatment and outcome of two adults with fibrogenesis imperfecta ossium are described. In this rare acquired disorder of bone, normal lamellar collagen is replaced by structurally unsound collagen-deficient tissue, which leads to extreme bone fragility and ununited fractures. Transmission microscopy and SEM showed striking ultrastructural changes in bone structure and mineralisation. Both patients had monoclonal IgG paraproteins in the plasma and one excreted monoclonal lambda light chains in the urine. No abnormal plasma cells were found in the bone marrow and there was no evidence of amyloid deposition in the tissues. In both patients initial treatment with 1 alpha-hydroxycholecalciferol appeared to be ineffective, but in one, repeated courses of melphalan and corticosteroids over three years together with 1 alpha-hydroxycholecalciferol produced striking clinical and histological improvement. The findings in these and other patients strongly suggest that paraproteinaemia is an integral feature of fibrogenesis imperfecta ossium, and this needs further investigation.

Six cases are reported in which a fibrosarcoma or malignant fibrous histiocytoma developed in relation to an enchondroma in a long bone. Four of the tumours were fibrosarcomata, and two were malignant fibrous histiocytomata. Five of the six cases were in the distal femur, which is a common site for old calcified enchondromata or "cartilage rests". The age of the patients was between fifty-six and eighty-six with a mean of seventy. Four were women, Five died less than one year after presentation. The fibrosarcomata and malignant fibrous histiocytomata do not appear to have arisen directly from the tumour cartilage but from the dense fibrous tissue surrounding necrotic areas in the enchondromata by a process analogous to that responsible for the development of fibrosarcomata in bone infarcts and chronic osteomyelitis. The possibility that some "dedifferentiated" chondrosarcomata are forms of collision tumour is discussed.

Two distinct lesions affect the articular cartilage of the patella. Surface degeneration occurs particularly on the odd facet; it is age dependent, often present in youth and it becomes more frequent with increasing age. It probably does not occasion patello-femoral pain in youth, but may predispose to degenerative arthritis in that joint in later years and is regarded as a consequence of habitual disuse. The term "basal degeneration" is used to describe a lesion in which there is a fasciculation of collagen in the middle and deep zones of cartilage without, at first, affecting the surface. It was found astride the ridge separating the medial from the odd facet in twenty-three adolescents who had complained of prolonged patello-femoral pain. They were treated by excision of the disc of affected cartilage, with relief of pain in most cases. The pathogenesis of basal degeneration is related to the functional anatomy of the patella.

The clinical features of eight patients with myositis ossificans progressiva are described and the effects of treatment with the diphosphonate EHDP, together with surgical removal of ectopic bone, are assessed. Early correct diagnosis remains unusual, mainly because the significance of the short great toes is unrecognised, and because myositis may be mistaken for bruising, sarcoma or mumps. The diphosphonate disodium etidronate (EDHP) was given to all patients in an attempt to suppress calcification of new lesions; in five of them ectopic bone was removed during the treatment. EHDP sometimes delayed the mineralisation of newly formed bone matrix after surgical removal but this delay could not be predicted. The variable effect of EHDP may depend particularly on the amount absorbed and on the activity of new bone formation.