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Keratosis follicularis spinulosa decalvans - Hair and Scalp
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Keratosis follicularis spinulosa decalvans - Hair and Scalp

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Contributors: Vivian Wong MD, PhD, Susan Burgin MD, Mary Gail Mercurio MD
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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.

For more information on X-linked KFSD, see OMIM.

For more information on autosomal dominant KFSD, see OMIM.

Codes

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

SNOMEDCT:
238626006 – Keratosis follicularis spinulosa decalvans

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 12/13/2016
Last Updated: 12/13/2016
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Keratosis follicularis spinulosa decalvans - Hair and Scalp
See also in: Overview
Print 9 Images
View all Images (9)
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Keratosis follicularis spinulosa decalvans : Photophobia, Corneal opacities, Erythema, Follicular configuration, Scarring alopecia, Eyelash loss, Entire scalp
Clinical image of Keratosis follicularis spinulosa decalvans
Copyright © 2018 VisualDx®. All rights reserved.