eMedicine Specialties > Dermatology > Pediatric Diseases

Ulerythema

Author: Aleksej Kansky, MD, PhD, Professor, Department of Dermatology, Ljubljana University Faculty of Medicine, Slovenia
Coauthor(s): Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Contributor Information and Disclosures

Updated: Jul 23, 2008

Introduction

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, Cornelia de Lange syndrome, and Rubinstein-Taybi syndrome.1,2,3 See Noonan Syndrome, de Lange Syndrome, and Rubinstein-Taybi Syndrome for more information on these topics.

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.

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.

Frequency

United States

The frequency is not known.

International

Worldwide incidence rates are not known.

Mortality/Morbidity

The disorder is completely benign; it does not endanger the patient's life.

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.

Clinical

History

  • The parents usually note erythema and tiny keratotic papules on the lateral parts of the eyebrows during early childhood. Similar lesions may develop on the lateral aspects of the front and on the cheeks.
  • Later, atrophy and loss of eyebrows may ensue. Sometimes, discrete follicular papules appear; follicular papules may also be present on the extensor sites of the arms and the thighs.

Physical

  • Erythema with slight follicular hyperkeratosis appears on the cheeks and lateral aspects of the eyebrows. The scalp may also be involved.
  • The affected areas may feel rough on delicate palpation.
  • A generalized facial erythema with scattered open and closed comedones and milia may be present. Rarely, similar lesions may be seen on the extensor surfaces of the arms and legs.
  • Hyperkeratotic follicular plugs with surrounding erythema that eventually evolves into coalescent follicular depressions in a honeycombed or worm-eaten pattern may be present in ulerythema of the cheeks, also known as atrophoderma vermiculatum. From the cheeks, ulerythema may extend to the ears and forehead.
  • The condition usually improves as the patient ages, but a loss of the lateral aspects of the eyebrows is possible, and scars may develop in the affected areas.

Causes

  • The true cause of ulerythema ophryogenes remains unknown. In a few families, this disorder is inherited in an autosomal dominant pattern. A molecular defect has so far not been elucidated, but certain clues are emerging as ulerythema appears as a trait in different syndromes.
  • Possible molecular defects are mentioned in ulerythema ophryogenes associated with more serious conditions such as Cornelia de Lange syndrome or Noonan syndrome, such as a deletion of the short arm of chromosome 18p caused by Y/18 translocation.4,5 Zouboulis et al even claim that the LAMA1 gene is involved6 ; however, Klein et al propose a 12q deletion as the cause of the condition.7
  • The Medscape Genomic Medicine Resource Center may be of interest.

More on Ulerythema

Overview: Ulerythema
Differential Diagnoses & Workup: Ulerythema
Treatment & Medication: Ulerythema
Follow-up: Ulerythema
References
[ CLOSE WINDOW ]

References

  1. Burnett JW, Schwartz MF, Berberian BJ. Ulerythema ophryogenes with multiple congenital anomalies. J Am Acad Dermatol. Feb 1988;18(2 Pt 2):437-40. [Medline].

  2. Gómez Centeno P, Rosón E, Peteiro C, Mercedes Pereiro M, Toribio J. Rubinstein--Taybi syndrome and ulerythema ophryogenes in a 9-year-old boy. Pediatr Dermatol. Mar-Apr 1999;16(2):134-6. [Medline].

  3. Snell JA, Mallory SB. Ulerythema ophryogenes in Noonan syndrome. Pediatr Dermatol. Mar 1990;7(1):77-8. [Medline].

  4. Nazarenko SA, Ostroverkhova NV, Vasiljeva EO, Nazarenko LP, Puzyrev VP, Malet P, et al. Keratosis pilaris and ulerythema ophryogenes associated with an 18p deletion caused by a Y/18 translocation. Am J Med Genet. Jul 16 1999;85(2):179-82. [Medline].

  5. Zouboulis CC, Stratakis CA, Rinck G, Wegner RD, Gollnick H, Orfanos CE. Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p. Pediatr Dermatol. Jun 1994;11(2):172-5. [Medline].

  6. Zouboulis CC, Stratakis CA, Gollnick HP, Orfanos CE. Keratosis pilaris/ulerythema ophryogenes and 18p deletion: is it possible that the LAMA1 gene is involved?. J Med Genet. Feb 2001;38(2):127-8. [Medline].

  7. Klein OD, Cotter PD, Schmidt AM, Bick DP, Tidyman WE, Albertson DG, et al. Interstitial deletion of chromosome 12q: genotype-phenotype correlation of two patients utilizing array comparative genomic hybridization. Am J Med Genet A. Nov 1 2005;138(4):349-54. [Medline].

  8. Handrick C, Alster TS. Laser treatment of atrophoderma vermiculata. J Am Acad Dermatol. Apr 2001;44(4):693-5. [Medline].

  9. Layton AM, Cunliffe WJ. A case of ulerythema ophryogenes responding to isotretinoin. Br J Dermatol. Nov 1993;129(5):645-6. [Medline].

  10. Azambuja R, Proença NG, Cardoso WV. [Ulerythema ophryogenes and folliculitis ulerythematosa reticulata]. Hautarzt. Jul 1987;38(7):411-3. [Medline].

  11. Zaba R, Bowszyc-Dmochowska M. Ulerythema ophryogenes. Postepy Dermatol. 1996;13:253-6.

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

ulerythema ophryogenes, keratosis pilaris rubra atrophicans faciei, folliculitis ulerythema reticulatum, honeycomb atrophy, atrophoderma vermiculatum

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Aleksej Kansky, MD, PhD, Professor, Department of Dermatology, Ljubljana University Faculty of Medicine, Slovenia
Aleksej Kansky, MD, PhD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

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, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

RELATED EMEDICINE ARTICLES
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.