Preferred Label : Progressive diaphyseal dysplasia;
ICD-11 definition : Camurati-Engelmann syndrome is a rare skeletal dysplasia of childhood onset and characterized
by generalized bone dysplasia with widening of the diaphyses of tubular bones. The
bones most frequently involved are the tibia, femur, humerus, ulna, radius, and the
base of the skull.;
ICD-11 synonym : diaphyseal sclerosis; Camurati-Engelmann syndrome; osteopathia hyperostotica multiplex infantis; diaphyseal dysplasia; Camurati-Engelmann disease;
ICD-11 inclusion : Camurati-Engelmann syndrome;
Origin ID : 1008460152;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Camurati-Engelmann syndrome is a rare skeletal dysplasia of childhood onset and characterized
by generalized bone dysplasia with widening of the diaphyses of tubular bones. The
bones most frequently involved are the tibia, femur, humerus, ulna, radius, and the
base of the skull.
