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Diseases

Discoid lupus erythematosus

OVERVIEW

  • Highlights & Basics
  • Images

DIAGNOSIS

  • Diagnostic Approach
  • Risk Factors
  • History & Exam
  • Tests
  • Differential Diagnosis
  • Criteria
  • Screening

TREATMENT

  • Tx Approach
  • Tx Options
  • Emerging Tx
  • Prevention

FOLLOW-UP

  • Overview
  • Complications

REFERENCES

  • Citations
  • Guidelines
  • Credits

PATIENT RESOURCES

  • Patient Instructions

Highlights & Basics

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Key Highlights
  • Discoid lupus erythematosus is the most common form of chronic cutaneous lupus erythematosus. Lesions are generally well-demarcated erythematous macules or papules with a scaly surface, which frequently evolve into larger coin-shaped plaques; typically neither pruritic nor painful.

  • Age of onset is usually between 20 and 40 years. Lesions are often precipitated or aggravated by ultraviolet light exposure; smoking is also considered a risk factor.

  • Over time, lesions slowly expand, producing areas of peripheral inflammation or hyperpigmentation, leaving a central region of scarring with telangiectasia and hypopigmentation. In hair-bearing areas, follicular plugging (scale at hair follicle) is often seen and scarring alopecia may be found.

  • Diagnosis is usually made on clinical grounds, but if in doubt is confirmed with a skin biopsy.

  • The mainstay of treatment for localized/limited disease is topical corticosteroids. Disseminated disease, or severe or refractory localized/limited disease, is treated with antimalarials, progressing to immunosuppressants if there is no response.

Skin biopsy showing hyperkeratosis, thinning of the epidermis, degeneration of the basal layer of th
Skin biopsy showing hyperkeratosis, thinning of the epidermis, degeneration of the basal layer of the epidermis (arrow), and a predominantly lymphocytic inflammatory cell infiltrate in the dermis
From the collection of Dr K. Blessing, used with permission

Quick Reference

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          Definition

          Epidemiology

          Etiology

          Pathophysiology

          content by BMJ Group
          Last updated

          Images

          • Skin biopsy showing hyperkeratosis, thinning of the epidermis, degeneration of the basal layer of th

            Skin biopsy showing hyperkeratosis, thinning of the epidermis, degeneration of the basal layer of the epidermis (arrow), and a predominantly lymphocytic inflammatory cell infiltrate in the dermis

          Citations

            Key Articles

            • O'Kane D, McCourt C, Meggitt S, et al; British Association of Dermatologists' Clinical Standards Unit. British Association of Dermatologists guidelines for the management of people with cutaneous lupus erythematosus 2021. Br J Dermatol. 2021 Dec;185(6):1112-23.[Abstract][Full Text]

            • Lu Q, Long H, Chow S, et al. Guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus. J Autoimmun. 2021 Sep;123:102707.[Abstract]

            • Kuhn A, Aberer E, Bata-Csörgő Z, et al. S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2017 Mar;31(3):389-404.[Abstract][Full Text]

            Referenced Articles

            • 1. Provost TT. The relationship between discoid and systemic lupus erythematosus. Arch Dermatol. 1994 Oct;130(10):1308-10.[Abstract]

            • 2. Healy E, Kieran E, Rogers S. Cutaneous lupus erythematosus - a study of clinical and laboratory prognostic factors in 65 patients. Ir J Med Sci. 1995 Apr-Jun;164(2):113-5.[Abstract]

            • 3. Pistiner M, Wallace DJ, Nessim S, et al. Lupus erythematosus in the 1980s: a survey of 570 patients. Semin Arthritis Rheum. 1991 Aug;21(1):55-64.[Abstract]

            • 4. Tebbe B. Clinical course and prognosis of cutaneous lupus erythematosus. Clin Dermatol. 2004 Mar-Apr;22(2):121-4.[Abstract]

            • 5. Siegel M, Lee SL. The epidemiology of systemic lupus erythematosus. Semin Arthritis Rheum. 1973;3(1):1-54.[Abstract]

            • 6. Santiago-Casas Y, Vilá LM, McGwin G Jr, et al. Association of discoid lupus with clinical manifestations and damage accrual in a multiethnic lupus cohort. Arthritis Care Res (Hoboken). 2012 May;64(5):704-12.[Abstract][Full Text]

            • 7. Gallego H, Crutchfield CE 3rd, Lewis EJ, et al. Report of an association between discoid lupus erythematosus and smoking. Cutis. 1999 Apr;63(4):231-4.[Abstract]

            • 8. Miot HA, Bartoli Miot LD, Haddad GR. Association between discoid lupus erythematosus and cigarette smoking. Dermatology. 2005;211(2):118-22.[Abstract]

            • 9. Knop J, Bonsmann G, Kind P, et al. Antigens of the major histocompatibility complex in patients with chronic discoid lupus erythematosus. Br J Dermatol. 1990 Jun;122(6):723-8.[Abstract]

            • 10. Seitz CS, Brocker EB, Trautmann A. Linear variant of chronic cutaneous lupus erythematosus: a clue for the pathogenesis of chronic cutaneous lupus erythematosus? Lupus. 2008 Dec;17(12):1136-9.[Abstract]

            • 11. Lin JH, Dutz JP, Sontheimer RD, et al. Pathophysiology of cutaneous lupus erythematosus. Clin Rev Allergy Immunol. 2007 Oct;33(1-2):85-106.[Abstract]

            • 12. O'Kane D, McCourt C, Meggitt S, et al; British Association of Dermatologists' Clinical Standards Unit. British Association of Dermatologists guidelines for the management of people with cutaneous lupus erythematosus 2021. Br J Dermatol. 2021 Dec;185(6):1112-23.[Abstract][Full Text]

            • 13. Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheumatol. 2019 Sep;71(9):1400-12.[Abstract][Full Text]

            • 14. Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002 Jul;20(3):373-85, v.[Abstract]

            • 15. Lu Q, Long H, Chow S, et al. Guideline for the diagnosis, treatment and long-term management of cutaneous lupus erythematosus. J Autoimmun. 2021 Sep;123:102707.[Abstract]

            • 16. Kuhn A, Aberer E, Bata-Csörgő Z, et al. S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2017 Mar;31(3):389-404.[Abstract][Full Text]

            • 17. Rahman P, Gladman DD, Urowitz MB. Smoking interferes with efficacy of antimalarial therapy in cutaneous lupus. J Rheumatol. 1998 Sep;25(9):1716-9.[Abstract]

            • 18. Kuhn A, Gensch K, Haust M, et al. Efficacy of tacrolimus 0.1% ointment in cutaneous lupus erythematosus: a multicenter, randomized, double-blind, vehicle-controlled trial. J Am Acad Dermatol. 2011 Jul;65(1):54-64, 64.e1-2.[Abstract]

            • 19. Barikbin B, Givrad S, Yousefi M, et al. Pimecrolimus 1% cream versus betamethasone 17-valerate 0.1% cream in the treatment of facial discoid lupus erythematosus: a double-blind, randomized pilot study. Clin Exp Dermatol. 2009 Oct;34(7):776-80.[Abstract]

            • 20. Sugano M, Shintani Y, Kobayashi K, et al. Successful treatment with topical tacrolimus in four cases of discoid lupus erythematosus. J Dermatol. 2006 Dec;33(12):887-91.[Abstract]

            • 21. Lampropoulos C, Sangle S, Harrison P, et al. Topical tacrolimus therapy of resistant cutaneous lesions in lupus erythematosus: a possible alternative. Rheumatology (Oxford). 2004 Nov;43(11):1383-5.[Abstract][Full Text]

            • 22. Tlacuilo-Parra A, Guevara-Gutierrez E, Gutierrez-Murillo F, et al. Pimecrolimus 1% cream for the treatment of discoid lupus erythematosus. Rheumatology (Oxford). 2005 Dec;44(12):1564-8.[Abstract][Full Text]

            • 23. Rothfield N, Sontheimer RD, Bernstein M. Lupus erythematosus: systemic and cutaneous manifestations. Clin Dermatol. 2006 Sep-Oct;24(5):348-62.[Abstract]

            • 24. Callen JP. Management of "refractory" skin disease in patients with lupus erythematosus. Best Pract Res Clin Rheumatol. 2005 Oct;19(5):767-84.[Abstract]

            • 25. Wenzel J, Brähler S, Bauer R, et al. Efficacy and safety of methotrexate in recalcitrant cutaneous lupus erythematosus: results of a retrospective study in 43 patients. Br J Dermatol. 2005 Jul;153(1):157-62.[Abstract]

            • 26. Goyal S, Nousari HC. Treatment of resistant discoid lupus erythematosus of the palms and soles with mycophenolate mofetil. J Am Acad Dermatol. 2001 Jul;45(1):142-4.[Abstract]

            • 27. Ruzicka T, Sommerburg C, Goerz G, et al. Treatment of cutaneous lupus erythematosus with acitretin and hydroxychloroquine. Br J Dermatol. 1992 Nov;127(5):513-8.[Abstract]

            • 28. Coburn PR, Shuster S. Dapsone and discoid lupus erythematosus. Br J Dermatol. 1982 Jan;106(1):105-6.[Abstract]

            • 29. Lindskov R, Reymann F. Dapsone in the treatment of cutaneous lupus erythematosus. Dermatologica. 1986;172(4):214-7.[Abstract]

            • 30. Stevens RJ, Andujar C, Edwards CJ, et al. Thalidomide in the treatment of the cutaneous manifestations of lupus erythematosus: experience in sixteen consecutive patients. Br J Rheumatol. 1997 Mar;36(3):353-9.[Abstract][Full Text]

            • 31. Pelle MT, Werth VP. Thalidomide in cutaneous lupus erythematosus. Am J Clin Dermatol. 2003;4(6):379-87.[Abstract]

            • 32. Cuadrado MJ, Karim Y, Sanna G, et al. Thalidomide for the treatment of resistant cutaneous lupus: efficacy and safety of different therapeutic regimens. Am J Med. 2005 Mar;118(3):246-50.[Abstract]

            • 33. Chasset F, Tounsi T, Cesbron E, et al. Efficacy and tolerance profile of thalidomide in cutaneous lupus erythematosus: a systematic review and meta-analysis. J Am Acad Dermatol. 2018 Feb;78(2):342-50.e4.[Abstract]

            • 34. Erceq A, Bovenschen HJ, van de Kerkhof PC, et al. Efficacy and safety of pulsed dye laser treatment for cutaneous discoid lupus erythematosus. J Am Acad Dermatol. 2009 Apr;60(4):626-32.[Abstract]

            • 35. Eskreis BD, Eng AM, Furey NL. Surgical excision of trauma-induced verrucous lupus erythematosus. J Dermatol Surg Oncol. 1988 Nov;14(11):1296-9.[Abstract]

            • 36. Trentin F, Tani C, Elefante E, et al. Treatment with Anifrolumab for discoid lupus erythematosus. JAMA Dermatol. 2023 Feb 1;159(2):224-6.[Abstract]

            • 37. Werth VP, Furie RA, Romero-Diaz J, et al. Trial of anti-BDCA2 antibody litifilimab for cutaneous lupus erythematosus. N Engl J Med. 2022 Jul 28;387(4):321-31.[Abstract][Full Text]

            • 38. American College of Rheumatology. 2022 American College of Rheumatology (ACR) guideline for vaccinations in patients with rheumatic and musculoskeletal diseases. Mar 2023 [internet publication].[Abstract][Full Text]

            • 39. Sulica VI, Kao GF. Squamous-cell carcinoma of the scalp arising in lesions of discoid lupus erythematosus. Am J Dermatopathol. 1988 Apr;10(2):137-41.[Abstract]

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