Camurati-engelmann disease: A late and rare sporadic case with vertebral and pelvic involvement

Camurati-Engelmann hastalığı ya da ilerleyici diafizyel displazi, nadir, otozomal dominant geçişli bir kemik hastalığıdır. Özellikle uzun kemiklerin diafizlerini etkileyen ilerleyici genişleme ve skleroz bu hastalığın karakteristik özelliğidir. Vertebra ve toraks kafesi tutulumu sadece ciddi vakalarda görülmektedir. Bu olguda direk grafi ve BT incelemelerinde, bilateral femur ve tibia diafizlerinde, vertebralarda, pelvis ve kostalarda simetrik genişleme ve sklerozis izlendi. Lomber BT incelemelerinde bu hastalık için tipik olan özellikle arka elemanlardaki tutulum dikkat çekici idi. Vertebra korpuslarının, kostaların ve pelvis kemiklerinin eş zamanlı ve belirgin tutulumu ile sporadik oluşu bu olguyu dikkat çekici kılmaktadır. Literatürde bu kadar diffüz tutulumla karakterize olguya rastlamadık.

Camurati-engelmann hastalığı: Vertebral ve pelvik tutulumla seyreden geç ve nadir sporadik olgu

Camurati-Engelmann disease (CED), or progressive diaphyseal dysplasia, is a rare, autosomal dominantly inherited bone disease. Progressive expansion and sclerosis predominantly affecting the diaphyses of the long bones are the characteristic hallmarks of this disease. The vertebral and thoracic-cage involvement is seen only in the most severe cases. This case is of interest because of its markedly involvement of vertebral bodies, ribs and pelvis at the same time, severe form and sporadic presentation. Also we were not able to find a report such a kind of diffuse involvement in the literature.

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