ECTS Abstracts (2015) 1 P47

Van Buchem's disease, a case report

Ilham Sehbani, Kawtar Nassar, Wafae Rachidi, Saadia Janani & Ouafa Mkinsi

Rheumatology Department, Ibn Rochd University Hospital, Casablanca, Morocco.

Van Buchem is a very rare disease. It is an uncommon autosomal recessive disorder, characterised by hyperostosis of the skull, mandible, clavicles, ribs, and diaphyseal cortices of the long bones. The establishment of accurate diagnosis of bone dysplasias when the abnormal findings are demonstrated only radiographically can be difficult. The dominant form of the disease most often symptomatically confined to the mandible, which help diagnosis. This leads to facial nerve pulsy, hearing loss, and optic atrophy. We report a 31 year-old Moroccan man showed marked radiographic changes throughout the skeleton and had a square pagetoid face.

Disclosure: The authors declared no competing interests.

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