Ulerythema Clinical Presentation

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 27, 2011
 

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.

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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.

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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.[5, 6] Zouboulis et al even claim that the LAMA1 gene is involved[7] ; however, Klein et al propose a 12q deletion as the cause of the condition.[8]

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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, University of Medicine and Dentistry of New Jersey-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

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.

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

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.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-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.

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.

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. Luria RB, Conologue T. Atrophoderma vermiculatum: a case report and review of the literature on keratosis pilaris atrophicans. Cutis. Feb 2009;83(2):83-6. [Medline].

  5. 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].

  6. 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].

  7. 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].

  8. Klein OD, Cotter PD, Schmidt AM, 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].

  9. Zegarska B, Kallas D, Schwartz RA, Czajkowski R, Uchanska G, Placek W. Graham-Little syndrome. Acta Dermatovenerol Alp Panonica Adriat. Oct 2010;19(3):39-42. [Medline].

  10. Dajani ZA, Kerns MJ, Mutasim DF. Unilateral keratosis pilaris atrophicans faciei mimicking follicular mucinosis. J Am Acad Dermatol. May 2011;64(5):e71-2. [Medline].

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

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

  13. Rodriguez-Lojo R, Pozo JD, Barja JM, et al. Keratosis pilaris atrophicans: treatment with intense pulsed light in four patients. J Cosmet Laser Ther. Aug 2010;12(4):188-90. [Medline].

 
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