Keratosis follicularis spinulosa decalvans in Child
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Synopsis
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.
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.
Codes
ICD10CM:
Q82.8 – Other specified congenital malformations of skin
SNOMEDCT:
238626006 – Keratosis follicularis spinulosa decalvans
Q82.8 – Other specified congenital malformations of skin
SNOMEDCT:
238626006 – Keratosis follicularis spinulosa decalvans
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Last Reviewed:12/12/2016
Last Updated:01/18/2022
Last Updated:01/18/2022
Keratosis follicularis spinulosa decalvans in Child
See also in: Hair and Scalp