AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

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, or Rubinstein-Taybi syndrome.

In 1889, Unna and Renzer coined the term ulerythema ophryogenes. 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 have been described. Its pathogenesis is unknown. 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

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

History

• The patient usually notes red coloration on the central part of the face, especially the cheeks and on eyebrows
• The patient may be troubled by the loss of lateral eyebrow hair later in life.

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 mentioned in connection with ulerythema ophryogenes are monosomy 18p and an 18p deletion caused by Y/18 translocation.

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

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

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Lab 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.

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical Care

The disease often improves with age. Successful treatment with carbon dioxide laser was reported in 2 patients, and the 585-nm pulsed dye lasers seemed efficient; however, in general, the treatment is rather unrewarding.

• Mild keratolytics, such as lactic acid 5%, urea 5%, or salicylic acid 2-5%, may be interchanged with cold creams, while low-potency corticosteroids may also be applied for short periods. Topical retinoids are worth of trying
• Sun protection creams are recommended when sun exposure is anticipated because frequent exposure to UV radiation exacerbates ulerythema ophryogenes. Local retinoids may not be effective.
• The risk-benefit correlation does not support the use of systemic retinoids for the treatment of ulerythema ophryogenes.

Diet

No special diet is necessary.

Activity

Excessive sunlight should be avoided.

FOLLOW-UP

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• Inpatient care is not needed for patients with ulerythema ophryogenes.

Further Outpatient Care

• Regular checkups are recommended for patients with this condition.

Transfer

• No transfer is required for patients with ulerythema ophryogenes.

Deterrence/Prevention

• Patients with ulerythema ophryogenes should not be exposed to the sun without UV protection.

Complications

• Scars may eventually develop at the sites of the hyperkeratotic papules.

Prognosis

• The disease tends to improve over time.

Patient Education

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

MISCELLANEOUS

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Although patients may want to sue their employer, they need to be aware that ulerythema ophryogenes is not believed to be an environmental or occupational disorder.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• References

• Azambuja R, Proenca NG, Cardoso WV. [Ulerythema ophryogenes and folliculitis ulerythematosa reticulata]. Hautarzt. Jul 1987;38(7):411-3. [Medline].
• 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].
• Gomez Centeno P, Roson E, Peteiro C, et al. Rubinstein--Taybi syndrome and ulerythema ophryogenes in a 9-year-old boy. Pediatr Dermatol. Mar-Apr 1999;16(2):134-6. [Medline].
• Handrick C, Alster TS. Laser treatment of atrophoderma vermiculata. J Am Acad Dermatol. Apr 2001;44(4):693-5. [Medline].
• Layton AM, Cunliffe WJ. A case of ulerythema ophryogenes responding to isotretinoin. Br J Dermatol. Nov 1993;129(5):645-6. [Medline].
• Nazarenko SA, Ostroverkhova NV, Vasiljeva EO, 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].
• Snell JA, Mallory SB. Ulerythema ophryogenes in Noonan syndrome. Pediatr Dermatol. Mar 1990;7(1):77-8. [Medline].
• Zaba R, Bowszyc-Dmochowska M. Ulerythema ophryogenes. Postepy Dermatol. 1996;13:253-6.
• Zouboulis CC, Stratakis CA, Rinck G, et al. Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p. Pediatr Dermatol. Jun 1994;11(2):172-5. [Medline].

Article Last Updated: Aug 10, 2005