Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences

View all Images (22)

Keratosis follicularis spinulosa decalvans in Child
See also in: Hair and Scalp
Other Resources UpToDate PubMed

Keratosis follicularis spinulosa decalvans in Child

See also in: Hair and Scalp
Contributors: Vivian Wong MD, PhD, Susan Burgin MD, Mary Gail Mercurio MD
Other Resources UpToDate PubMed

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.

Codes

ICD10CM:
Q82.8 – Other specified congenital malformations of skin

SNOMEDCT:
238626006 – Keratosis follicularis spinulosa decalvans

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Follicular papules on the extremities should lead to a consideration of pityriasis rubra pilaris, which may start in childhood.

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:12/12/2016
Last Updated:01/18/2022
Copyright © 2022 VisualDx®. All rights reserved.
Keratosis follicularis spinulosa decalvans in Child
See also in: Hair and Scalp
A medical illustration showing key findings of Keratosis follicularis spinulosa decalvans : Photophobia, Corneal opacities, Erythema, Follicular configuration, Scarring alopecia, Eyelash loss, Entire scalp
Clinical image of Keratosis follicularis spinulosa decalvans - imageId=1169189. Click to open in gallery.  caption: 'Diffuse scarring alopecia of the scalp with patchy erythema of the scalp, ear, and cheek.'
Diffuse scarring alopecia of the scalp with patchy erythema of the scalp, ear, and cheek.
Copyright © 2022 VisualDx®. All rights reserved.