A unitary hypothesis is offered to explain the various proliferative processes occurring around the phalanges. In the past, these have been separately designated as proliferative periostitis, bizarre parosteal osteochondromatous proliferation, and turret exostosis. Because the appearances of these entities depend on temporal factors, breaching of the periosteum, and local anatomic features, we suggest a single term, proliferative periosteal processes of phalanges.
Neonatal onset multisystem inflammatory disease is a rare disorder first described by Lorber in 1973. An additional 29 cases have been recorded. Two patients are described here, one with a 17 year follow-up. The typical features are a rash, fever, adenopathy, hepatosplenomegaly, and a severe, deforming arthropathy predominantly affecting large joints. The most striking feature is the onset in the neonatal period. Other associated features include inflammation, chronic meningitis, anemia, and persistent leukocytosis. Most, if not all, patients develop bizarre epiphyseal radiographic findings that are virtually pathognomonic. This disease is distinct from Still disease.
A 55-year-old man with the classical mucocutaneous lesions of xanthoma disseminatum has been followed up for a period of 13 years. The special features of this case, which make it unique, are as follows: (1) the availability of histologic data on multiple lesions for more than a ten-year period; (2) the progressive nature of the multiple osseous lesions; (3) the metabolic studies that show no evidence for accumulation of abnormal sterols in a xanthoma, the blood, or intestinal aspirate; (4) the development of hypothyroidism and symptoms or signs, or both, of an intracerebral and an intraspinal lesion; (5) the partial regression of the cutanous symptoms and lesions while receiving clofibrate, in spite of progression of the mucous membrane and osseous lesions, and (6) the failure to develop diabetes insipidus to date.
On the basis of published anatomical and skeletal anthropological surveys supplemented by published and unpublished commissioned radiologic surveys, the distribution of coracoclavicular joints in the world is described. The findings are in keeping with a focus having developed in central Asia in Pleistocene times. Thus, this anatomical variant can serve, by regression, as a marker for population migration from prehistoric times to the present.
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