We report a family with diffuse idiopathic skeletal hyperostosis (DISH). The most striking occurrence was severe cervical disease without extensive dorsal involvement. From the tissue typing results of our two sibling patients, it appears less likely that, if there is a hereditary component, it is linked to HLA status. It remains to be seen whether this is a new disease entity or an unusual familial variant of DISH. We are unaware of a similar published report.

A 23 year old man was referred with a painful stiff neck of 3 years’ duration. On examination, all movements of his cervical spine were restricted. Other spinal movements were normal. Inflammatory markers were normal and HLA-B27 was negative. An x ray examination of the sacroiliac joints, thoracic and lumbar spine were unremarkable. However, the cervical spine radiograph showed gross anterior osteophytosis (fig 1A⇓).