Ulerythema

Updated: Jul 18, 2017
  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Ulerythema ophryogenes, a rare cutaneous disorder, is characterized by inflammatory keratotic facial papules that may result in scars, atrophy, and alopecia. This disorder has been described in association with other congenital anomalies such as Noonan syndrome, de Lange syndrome, and Rubinstein-Taybi syndrome. [1, 2, 3, 4] Ulerythema ophryogenes falls within the broader category of keratosis pilaris atrophicans. [5] It was suggested to link this category with lichen planopilaris under the rubric of an even broader grouping, lichenoid folliculitis. [6]

In 1889, Tancer introduced the term ulerythema ophryogenes. In 1892, Unna coined the term. In 1925, Gans used the designation keratosis pilaris rubra atrophicans faciei.

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Pathophysiology

Ulerythema ophryogenes is a relatively infrequent disorder that mainly affects children and young adults. It is usually sporadic, although cases inherited in an autosomal dominant pattern also are described. Although the condition is benign, it is still worrisome for parents. The inclusion of ulerythema ophryogenes into the concept of pilar keratoses has been proposed. Griffiths proposes the inclusion into the broader concept of keratosis pilaris atrophicans, while Dawber proposes the inclusion into scarring follicular keratosis.

Genetic factors are important. There appears to be an association between the syndrome of partial monosomy of the short arm of chromosome 18 and the genodermatoses keratosis pilaris and ulerythema ophryogenes, [7] which could be viewed as a new rare syndrome. [8] A few patients have been described, suggesting this linkage. The genes responsible for follicular keratinization could be located on the short arm of this chromosome. Most patients with cardiofaciocutaneous syndrome exhibit keratosis pilaris and ulerythema ophryogenes. [9]

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Epidemiology

Frequency

Worldwide incidence rates are not known.

Race

Individuals of all races can be affected.

Sex

This condition can affect males and females.

Age

Children and young adults are most commonly affected with this condition.

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Prognosis

The disease tends to improve over time. However, this disorder may be the first sign of Noonan syndrome, which is important to recognize owing to its cardiovascular defects. [10]

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Patient Education

Young patients should be encouraged to live with this mild esthetic shortcoming.

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