Caffey Disease

Caffey disease is an autosomal dominant disorder characterized by an infantile episode of massive subperiosteal bone neoplasm typically affecting the long bone diaphyses, mandible, and clavicles. Painful swelling and systemic fever often accompany the episode, which usually begins before 5 months of age and resolves before 2 years of age.

Radiologically, a partly very florid periosteal neoplasm of bone is recognizable, which results in a considerable thickening of the cortical bone.

Laboratory findings include an elevated alkaline phosphatase level and sometimes an increase in white blood cell count and erythrocyte sedimentation rate. Recurrent relapses are uncommon.

Treatment is palliative analgesic. Success with high-dose immunoglobulins has been reported. Corticosteroids have been given to accelerate bone remodeling.