Confirmation of X-Linked Inheritance and Provisional Mapping of the Keratosis Follicularis Spinulosa Decalvans Gene on XP in a Large Dutch Family

Abstract
In a large Dutch family with keratosis follicularis spinulosa decalvans (KFSD, MIM 308800), DNA linkage analysis was performed in order to locate the gene. Pedigree analysis and lod score calculation confirmed X-linked inheritance and revealed significant linkage to DNA markers on Xp. A maximum lod score of 5.70 at 0 = 0.0 was obtained with DXS41 (p99.6). The KFSD gene is tentatively located on Xp21.2–p22.2.