Osteopathia striata with cranial sclerosis: Literature reappraisal argues for X-linked inheritance

Osteopathia striata with cranial sclerosis: Literature reappraisal argues f or X-linked inheritance: Osteopathia striata with cranial sclerosls (OS-CS) is characterized by linear striations of tubular bones and fan-shaped conf igurations of the ilia. Although referred in literature as an autosomal dom inant disorder the clinical pattern of partial involvement as well as the r eported family observations plead for X-linked inheritance with mild striat ed bone affections in carrier women and severe syndromic morbidity and high mortality in the males. Sporadic affected males are probably somatic mosai cs. There is no proven rather-son transmission. Symptomatic osteopathia str iata (OS) is characteristic in X-linked focal dermal hypoplasia Goltz-Gorli n.