Keratosis follicularis spinulosa decalvans (KFSD) is a rare, progressive, cicatricial (scarring) alopecia disease closely related to keratosis atrophicans faciei, keratosis pilaris atrophicans, and atrophoderma vermiculatum. X-linked dominant, autosomal dominant, and sporadic cases have been reported.

KFSD begins in early childhood with keratosis pilaris on the face, which progresses to the trunk and extremities. Limited or widespread cicatricial alopecia of the scalp, eyelashes, and eyebrows ensues. Boys may have corneal dystrophy with associated photophobia. A pustular variant with crusting and scaling on the scalp may occur around puberty. The overall presentation in girls in X-linked dominant cases may be milder than in boys.