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Ulerythema

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 24, 2016
 

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]

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,[6] which could be viewed as a new rare syndrome.[7] 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.[8]

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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.[9]

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Patient Education

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

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Contributor Information and Disclosures
Author

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

James J Nordlund, MD Professor Emeritus, Department of Dermatology, University of Cincinnati College of Medicine

James J Nordlund, MD is a member of the following medical societies: American Academy of Dermatology, Sigma Xi, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Aleksej Kansky, MD, PhD, to the development and writing of this article.

References
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