Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences

View all Images (13)

Keratosis pilaris atrophicans faciei in Adult
Other Resources UpToDate PubMed

Keratosis pilaris atrophicans faciei in Adult

Contributors: Amy Fox MD, David Dasher MD, Jeffrey D. Bernhard MD
Other Resources UpToDate PubMed

Synopsis

Keratosis pilaris atrophicans faciei (KPAF), also known as ulerythema ophryogenes, is a disorder of follicular keratinization presenting in infancy. Inheritance is thought to be autosomal dominant. Affected individuals will have hyperkeratotic papules along eyebrows and cheeks beginning in the first few months of life, which can progress into scarring alopecia. Thinning or loss of the lateral portion of the eyebrows is a common clinical finding and is often seen in conjunction with traditional keratosis pilaris involving arms and legs. Progression of the disease ceases at puberty, but sequelae are often permanent.

The condition is benign and of unknown cause. It is usually diagnosed in childhood.

KPAF has been described in patients with woolly hair, Noonan syndrome, cardiofaciocutaneous syndrome, Rubinstein-Taybi syndrome, and Cornelia de Lange syndrome. These patients will have additional features suggestive of these syndromes and may require work-up and genetic evaluation.

Codes

ICD10CM:
Q84.2 – Other congenital malformations of hair

SNOMEDCT:
400126005 – Ulerythema ophryogenes

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

To perform a comparison, select diagnoses from the classic differential

Subscription Required

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Updated:09/25/2017
Copyright © 2024 VisualDx®. All rights reserved.
Keratosis pilaris atrophicans faciei in Adult
A medical illustration showing key findings of Keratosis pilaris atrophicans faciei : Cheek, Erythema, Follicular configuration, Sparse eyebrows
Clinical image of Keratosis pilaris atrophicans faciei - imageId=692642. Click to open in gallery.  caption: 'Marked erythema of the cheek and ear with follicular prominence on the cheek.'
Marked erythema of the cheek and ear with follicular prominence on the cheek.
Copyright © 2024 VisualDx®. All rights reserved.