INCIDENTAL FINDING OF A PROGRESSIVE DIAPH YSEAL DYSPLASIA (CAMURATI-ENGELMANN SYNDROME)

In a 35 year-old female diaphyseal hyperostoses of femur and tibia ind icative of a Camurati-Engelmann syndrome (progressive diaphyseal dyspl asia) were discovered during routine phlebography. This rare disorder belongs to the group of osteochondrodysplasias and affects primarily t he diaphyses of long bones. Sporadic as well as familial cases have be en observed. Although the cause and pathogenetic mechanism of the dise ase are unknown, an autosomal dominant inheritance is being discussed. Diagnosis was based on radiological findings, which are presented alo ng with the scintigraphic appearance of the disease.