Osteopathia striata-pigmentary dermopathy-white forelock syndrome
ORPHA:2779· ICD-10 Q77.8
Definition
A rare primary bone dysplasia characterized by the association of osteopathia striata (longitudinal striations through most of the long bones) with a macular, hyperpigmented dermopathy and a white forelock.
- Prevalence
- <1 / 1 000 000
- Inheritance
- Autosomal dominant, X-linked dominant
- Age of onset
- Neonatal