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Keratosis pilaris atrophicans faciei in Child
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Keratosis pilaris atrophicans faciei in Child

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Contributors: Amy Fox MD, David Dasher MD, Jeffrey D. Bernhard MD
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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

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Last Updated: 09/26/2017
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Keratosis pilaris atrophicans faciei in Child
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Keratosis pilaris atrophicans faciei : Cheek, Erythema, Follicular configuration, Sparse eyebrows
Clinical image of Keratosis pilaris atrophicans faciei
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