eMedicine Specialties > Dermatology > Pediatric Diseases

Ulerythema: Differential Diagnoses & Workup

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

Differential Diagnoses

Ichthyosis Vulgaris, Hereditary and Acquired

Other Problems to Be Considered

Differentiate the following:

  • Atrophoderma vermiculatum
  • Keratosis pilaris atrophicans (ulerythema ophryogenes) and keratosis pilaris decalvans
  • Psoriasis, atopic dermatitis, pityriasis rubra pilaris, and ichthyosis vulgaris
Ulerythema is not always differentiated from keratosis pilaris, which is rather common in young women; keratosis pilaris commonly occurs on the extensor surfaces of the arms. Keratosis follicularis spinulosa decalvans mainly affects the scalp, but it should also be considered a differential diagnosis.

Pityriasis rubra pilaris and psoriasis may also require distinction. Although pityriasis rubra pilaris may be similar to psoriasis, important clinical symptoms of psoriasis are missing, including the whitish scales, the Auspitz sign, and the nail pitting. The tiny follicular yellow-red papules are a typical clinical sign of pityriasis rubra pilaris, thereby distinguishing it from psoriasis.

In small children, ulerythema must be differentiated from seborrhoic dermatitis and from atopic dermatitis.

Workup

Laboratory Studies

No laboratory test, except histopathologic examination, is diagnostic.

Histologic Findings

In the early phase, pilosebaceous follicles filled with keratotic plugs and a mild perifollicular inflammatory infiltrate are observed. Cystic dilatation of the hair follicles may be evident on the cheeks. Later, atrophy of both hair follicles and sebaceous glands, as well as a dermal fibrosis, may appear.

Staging

Two stages may eventually be observed: an early stage characterized by follicular hyperkeratosis and a late stage with some evidence of fibrosis and atrophy.

More on Ulerythema

Overview: Ulerythema
Differential Diagnoses & Workup: Ulerythema
Treatment & Medication: Ulerythema
Follow-up: Ulerythema
References
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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.

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Further Reading

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Keywords

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

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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.

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