Steel hair syndrome is
[A] Scleroderma
[B] DLE
[C] Menkes disease
[D] Osteogenesis imperfecta
Menkes disease
- Menkes disease is characterized by an unstable body temperature and strikingly tangled hair, which is colorless or steel-colored, sparse, and known to break easily.
- X-linked disorder of intestinal copper absorption.
- Metabolic defect – The third phase: Transport across the serosal aspect of the mucosal cell membrane is defective.
- Defect at molecular level – Mutations in the gene for a “copper binding P type ATPase
