Keratosis follicularis spinulosa decalvans

Abstract

A case of keratosis follicularis spinulosa decalvans in young Kashmiri male is presented. This rare disorder with genetic and clinical heterogeneity, inherited in an X-linked recessive pattern is secondary to mutation in SAT1 gene located at Xp22.1. Although multigenerational families with male to male transmission suggestive of autosomal dominant inheritance have been reported, we present a case with no family history.