Home Progressive Diaphyseal disease: Camurati-Engelmann

Progressive Diaphyseal disease: Camurati-Engelmann

Types

Defining Characteristics

Gradual appearance of symmetric hyperostosis on periosteal and endosteal surfaces of long bones

Disease Development

Mutation in TGF-B1, a latency protein that is a normally a chronic sequesterer and inhibitor of TGF-B in bone

Potential Causes

Unregulated TGF-B causes overstimulation of osteoblasts and excessive bone formation

Epidemiology

Variable age, severity, course

Conventional Treatment

Glucocorticoids for pain relief