[Course of diaphyseal dysplasia. Camurati-Engelmann disease followed for 14 years].

The authors report a case of a child with Camurati-Engelmann disease de Camurati-Engelmann followed-up for 14 years. The diagnosis was made at the age of 5 years, with a typical picture. During the course of the disease, certain discordant facts emerged with, in particular, the appearance of osteoporotic lesions in the pelvis, metaphyses, epiphyses and skull. This led to the possibility of other diagnoses being envisaged but none could be made definitely. Furthermore, the osteoporotic lesions could not be explained by corticosteroid therapy which was taken in too irregular a manner. Is there a borderline disease within the broad definition of diaphyseal dysplasia?