Discoid Lupus Erythematosus: Background, Etiology, Epidemiology 16/04/17 22)07

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Discoid Lupus Erythematosus

Updated: Feb 26, 2016
Author: A Brooke W Eastham, MD; Chief Editor: William D James, MD more...

OVERVIEW

Background
Cutaneous lupus erythematosus (CLE) can be divided into 3 main subtypes: acute, subacute,
and chronic, all of which demonstrate photosensitivity. Acute cutaneous lupus erythematosus
(ACLE) most commonly presents as symmetric erythema overlying the malar cheeks and nasal
bridge with sparing of the nasolabial folds (butterfly rash). However, it can also present as a
diffuse morbilliform eruption with erythema and edema of the hands, with prominent sparing of
the joints. Subacute cutaneous lupus erythematosus (SCLE) characteristically presents as
annular or psoriasiform plaques in a photodistribution. Chronic cutaneous lupus erythematosus
(CCLE) can be further divided into 3 main types: discoid lupus erythematosus (DLE), tumid
lupus, and lupus panniculitis. Tumid lupus typically presents with juicy papules and plaques that
heal without scarring, whereas lupus panniculitis involves the subcutaneous tissue, leading to
painful subcutaneous nodules that heal with depression and atrophy.

DLE classically presents with erythematous-to-violaceous, scaly plaques with prominent

follicular plugging that often results in scarring and atrophy (see the images below). DLE may
occur in the absence of systemic disease, or it may occur in association with systemic lupus
erythematosus (SLE).

Discoid lupus erythematosus on the face.

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Chronic scarred lesion of discoid lupus erythematosus.

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The risk of progression to SLE in patients with DLE was demonstrated to be higher than
previously reported (16.7% progression within 3 years of diagnosis, as compared with previous
data indicating that < 5-10% of patients with DLE progress to SLE). [1, 2] Overall, patients with
DLE rarely fulfill 4 or more of the 11 American College of Rheumatology (ACR) criteria used to
classify SLE. [3] Serologic abnormalities are uncommon.

Therapy with sunscreens, topical corticosteroids, and antimalarial agents is often effective. (See
Clinical and Treatment.) However, immunosuppressive and/or immunomodulatory agents may
be required for recalcitrant disease.

Patient education
Instruct patients in sun-avoidance techniques and the proper use of sunscreens, hats, and
protective clothing. Advise patients to quit smoking. Discuss the possibility of systemic
involvement with patients. Pamphlets from the Lupus Foundation of America, Inc and JAMA
Dermatology Cutaneous Lupus Patient Page [4] concerning skin disease and photosensitivity are
useful adjuncts to verbally delivered information. For patient education information, see the
Arthritis Center. Also see Prognosis and Treatment.

Etiology
Lupus erythematosus is a polygenic autoimmune disease linked to various HLA subtypes,
immune signaling, and environmental factors, which ultimately leads to autoantibody production
and T-cell dysfunction. However, the exact etiology of discoid lupus erythematosus (DLE) is not
well understood. DLE likely occurs in genetically predisposed individuals, but the exact genetic
connection has not been determined. It has been suggested that a heat-shock protein is induced
in the keratinocyte following ultraviolet (UV) light exposure or stress, and this protein may act as
a target for gamma (delta) T-cellmediated epidermal cell cytotoxicity. Additionally, toll-like
receptors may be involved in the pathogenesis. [5]

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Epidemiology
Worldwide, the prevalence of systemic lupus erythematosus (SLE) ranges from 17-48 cases per
100,000 population. The highest prevalence of SLE occurs in persons aged 40-60 years, with
SLE onset most often occurring in patients in their 20s and 30s. SLE is approximately 10 times
more common in women than in men. In a 2009 study from Olmstead County, Minnesota,
Durosaro et al demonstrated that the incidence of cutaneous lupus erythematosus (CLE) is
comparable to that of SLE. [6]

Discoid lupus erythematosus (DLE) is responsible for 50-85% of cases of CLE and occurs 2-3
times more frequently in women than in men. DLE is slightly more common in African Americans
than in whites or Asians. Although DLE may occur at any age, it most often develops in persons
aged 20-40 years.

Prognosis
Although the prognosis of patients with discoid lupus erythematosus (DLE) is favorable
regarding mortality, morbidity can be considerable. Patients may experience pain or burning of
their lesions, and many experience disfigurement from the scars or atrophy that can develop.
Scarring alopecia is particularly disturbing for patients. Prompt treatment of early lesions may
help prevent or lessen the severity of scarring and atrophy.

Exacerbation is common with increased sun exposure, particularly in the spring and summer.
Serious systemic disease is rare, but when it occurs, patients may develop life-altering
sequelae. Malignant degeneration within DLE lesions is uncommon. However, prompt biopsy of
suggestive lesions developing within chronic DLE lesions is warranted. [7]

Clinical Presentation

References

1. Prystowsky SD, Gilliam JN. Discoid lupus erythematosus as part of a larger disease
spectrum. Correlation of clinical features with laboratory findings in lupus erythematosus.
Arch Dermatol. 1975 Nov. 111(11):1448-52. [Medline].

2. Grnhagen CM, Fored CM, Granath F, Nyberg F. Cutaneous lupus erythematosus and the
association with systemic lupus erythematosus: a population-based cohort of 1088
patients in Sweden. Br J Dermatol. 2011 Jun. 164(6):1335-41. [Medline].

3. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of
systemic lupus erythematosus. Arthritis Rheum. 1982 Nov. 25(11):1271-7. [Medline].

4. Eastham AB, Vleugels RA. Cutaneous lupus erythematosus. JAMA Dermatol. 2014 Mar.
150(3):344. [Medline].

5. Lehmann P, Hlzle E, Kind P, Goerz G, Plewig G. Experimental reproduction of skin

lesions in lupus erythematosus by UVA and UVB radiation. J Am Acad Dermatol. 1990
Feb. 22(2 Pt 1):181-7. [Medline].

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Discoid Lupus Erythematosus: Background, Etiology, Epidemiology 16/04/17 22)07

6. Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence of cutaneous lupus
erythematosus, 1965-2005: a population-based study. Arch Dermatol. 2009 Mar.
145(3):249-53. [Medline].

7. Tao J, Zhang X, Guo N, et al. Squamous cell carcinoma complicating discoid lupus
erythematosus in Chinese patients: review of the literature, 1964-2010. J Am Acad
Dermatol. 2012 Apr. 66(4):695-6. [Medline].

8. Merola JF, Prystowsky SD, Iversen C, Gomez-Puerta JA, Norton T, Tsao P, et al.
Association of discoid lupus erythematosus with other clinical manifestations among
patients with systemic lupus erythematosus. J Am Acad Dermatol. 2013 Jul. 69 (1):19-24.
[Medline].

9. Parish LC, Kennedy RJ, Hurley J. Palmar lesions in lupus erythematosus. Arch Dermatol.
1967 Sep. 96(3):273-6. [Medline].

10. Spann CR, Callen JP, Klein JB, Kulick KB. Clinical, serologic and immunogenetic studies
in patients with chronic cutaneous (discoid) lupus erythematosus who have verrucous
and/or hypertrophic skin lesions. J Rheumatol. 1988 Feb. 15(2):256-61. [Medline].

11. Martens PB, Moder KG, Ahmed I. Lupus panniculitis: clinical perspectives from a case
series. J Rheumatol. 1999 Jan. 26(1):68-72. [Medline].

12. Jung H, Bobba R, Su J, et al. The protective effect of antimalarial drugs on

thrombovascular events in systemic lupus erythematosus. Arthritis Rheum. 2010 Mar.
62(3):863-8. [Medline].

13. Petri M. Use of hydroxychloroquine to prevent thrombosis in systemic lupus erythematosus

and in antiphospholipid antibody-positive patients. Curr Rheumatol Rep. 2011 Feb.
13(1):77-80. [Medline].

14. Callen JP. Cutaneous lupus erythematosus: a personal approach to management.

Australas J Dermatol. 2006 Feb. 47(1):13-27. [Medline].

15. James JA, Kim-Howard XR, Bruner BF, et al. Hydroxychloroquine sulfate treatment is
associated with later onset of systemic lupus erythematosus. Lupus. 2007. 16(6):401-9.
[Medline].

16. Wahie S, Daly AK, Cordell HJ, et al. Clinical and pharmacogenetic influences on response
to hydroxychloroquine in discoid lupus erythematosus: a retrospective cohort study. J
Invest Dermatol. 2011 Oct. 131(10):1981-6. [Medline].

17. Atra E, Sato EI. Treatment of the cutaneous lesions of systemic lupus erythematosus with
thalidomide. Clin Exp Rheumatol. 1993 Sep-Oct. 11(5):487-93. [Medline].

18. Coelho A, Souto MI, Cardoso CR, et al. Long-term thalidomide use in refractory cutaneous
lesions of lupus erythematosus: a 65 series of Brazilian patients. Lupus. 2005. 14(6):434-
9. [Medline].

19. Shah A, Albrecht J, Bonilla-Martinez Z, et al. Lenalidomide for the treatment of resistant
discoid lupus erythematosus. Arch Dermatol. 2009 Mar. 145(3):303-6. [Medline]. [Full
Text].

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Discoid Lupus Erythematosus: Background, Etiology, Epidemiology 16/04/17 22)07

20. Marmor MF, Kellner U, Lai TY, Lyons JS, Mieler WF. Revised recommendations on
screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmology. 2011 Feb.
118(2):415-22. [Medline].

21. Wahie S, Daly AK, Cordell HJ, et al. Clinical and pharmacogenetic influences on response
to hydroxychloroquine in discoid lupus erythematosus: a retrospective cohort study. J
Invest Dermatol. 2011 Oct. 131(10):1981-6. [Medline].

22. Gallego H, Crutchfield CE 3rd, Lewis EJ, Gallego HJ. Report of an association between
discoid lupus erythematosus and smoking. Cutis. 1999 Apr. 63(4):231-4. [Medline].

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

24. Lipsker D, Boeckler P, Hauptmann G. Cigarette smoking and lupus erythematosus.

Dermatology. 2006. 212(3):262. [Medline].

25. Piette EW, Foering KP, Chang AY, et al. Impact of smoking in cutaneous lupus
erythematosus. Arch Dermatol. 2012 Mar. 148(3):317-22. [Medline]. [Full Text].

26. Wenzel J, Brhler S, Bauer R, Bieber T, Tting T. 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. [Medline].

27. Callen JP, Spencer LV, Burruss JB, Holtman J. Azathioprine. An effective, corticosteroid-
sparing therapy for patients with recalcitrant cutaneous lupus erythematosus or with
recalcitrant cutaneous leukocytoclastic vasculitis. Arch Dermatol. 1991 Apr. 127(4):515-22.
[Medline].

28. Gammon B, Hansen C, Costner MI. Efficacy of mycophenolate mofetil in antimalarial-

resistant cutaneous lupus erythematosus. J Am Acad Dermatol. 2011 Oct. 65(4):717-21.
[Medline].

29. Kreuter A, Tomi NS, Weiner SM, Huger M, Altmeyer P, Gambichler T. Mycophenolate
sodium for subacute cutaneous lupus erythematosus resistant to standard therapy. Br J
Dermatol. 2007 Jun. 156(6):1321-7. [Medline].

30. Erceg A, Bovenschen HJ, van de Kerkhof PC, de Jong EM, Seyger MM. Efficacy and
safety of pulsed dye laser treatment for cutaneous discoid lupus erythematosus. J Am
Acad Dermatol. 2009 Apr. 60(4):626-32. [Medline].

31. Chang AY, Piette EW, Foering KP, Tenhave TR, Okawa J, Werth VP. Response to
antimalarial agents in cutaneous lupus erythematosus: a prospective analysis. Arch
Dermatol. 2011 Nov. 147(11):1261-7. [Medline]. [Full Text].

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

33. Duong DJ, Spigel GT, Moxley RT 3rd, Gaspari AA. American experience with low-dose
thalidomide therapy for severe cutaneous lupus erythematosus. Arch Dermatol. 1999 Sep.
135(9):1079-87. [Medline].

http://emedicine.medscape.com/article/1065529-overview#aw2aab6b2b2 Pgina 5 de 8
Discoid Lupus Erythematosus: Background, Etiology, Epidemiology 16/04/17 22)07

34. Housman TS, Jorizzo JL, McCarty MA, Grummer SE, Fleischer AB Jr, Sutej PG. Low-dose
thalidomide therapy for refractory cutaneous lesions of lupus erythematosus. Arch
Dermatol. 2003 Jan. 139(1):50-4. [Medline].

35. Newton RC, Jorizzo JL, Solomon AR Jr, et al. Mechanism-oriented assessment of
isotretinoin in chronic or subacute cutaneous lupus erythematosus. Arch Dermatol. 1986
Feb. 122(2):170-6. [Medline].

36. Tsianakas A, Herzog S, Landmann A, Patsinakidis N, Perusqua Ortiz AM, Bonsmann G,

et al. Successful treatment of discoid lupus erythematosus with fumaric acid esters. J Am
Acad Dermatol. 2014 Jul. 71(1):e15-7. [Medline].

37. Debu A, Girard C, Bessis D. Discoid lupus erythematosus successfully treated by

photodynamic therapy. Br J Dermatol. 2014 Sep 12. [Medline].

38. Turan E, Sinem Bagci I, Turgut Erdemir A, Salih Gurel M. Successful treatment of
generalized discoid lupus erythematosus with imiquimod cream 5%: a case report and
review of the literature. Acta Dermatovenerol Croat. 2014. 22(2):150-9. [Medline].

39. Kindle SA, Wetter DA, Davis MD, Pittelkow MR, Sciallis GF. Lenalidomide treatment of
cutaneous lupus erythematosus: the Mayo Clinic experience. Int J Dermatol. 2016 Feb 12.
3 (4):883-99. [Medline].

40. Okon L, Rosenbach M, Krathen M, Rose M, Propert K, Okawa J, et al. Lenalidomide in

treatment-refractory cutaneous lupus erythematosus: Efficacy and safety in a 52-week
trial. J Am Acad Dermatol. 2014 Mar. 70 (3):583-4. [Medline].

Media Gallery

Discoid lupus erythematosus on the face.

Chronic scarred lesion of discoid lupus erythematosus.
Lesions of discoid lupus erythematosus in the conchal bowl demonstrate patulous follicles
with follicular plugging.
Palmar lesions of discoid lupus erythematosus.
Scarring alopecia of discoid lupus erythematosus.
Widespread scarring alopecia.
Hypertrophic lesions of chronic cutaneous lupus erythematosus on the dorsal hands.
Characteristic lesions were observed elsewhere.

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Contributor Information and Disclosures

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Author

A Brooke W Eastham, MD Board Certified Dermatologist, Nashville Skin and Cancer

A Brooke W Eastham, MD is a member of the following medical societies: Alpha Omega Alpha,
American Academy of Dermatology, Medical Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School;
Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate
Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega
Alpha, American Academy of Dermatology, American College of Rheumatology, American
Medical Association, Society for Investigative Dermatology, Medical Dermatology Society,
Dermatology Foundation

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology,

University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha,
American Academy of Dermatology, American College of Physicians, American College of
Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA;
Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from
UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor
and intermittent author; Received royalty from Elsevier for book author/editor; Received
dividends from trust accounts, but I do not control these accounts, and have directed our
managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various
trust accounts include some pharmaceutical companies and device makers for i inherited these
trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories;
AbbVie; Procter and Gamble; Amgen.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System;
Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair,
Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical

Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of
Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery,
Phi Beta Kappa

Disclosure: Nothing to disclose.

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Discoid Lupus Erythematosus: Background, Etiology, Epidemiology 16/04/17 22)07

Julia R Nunley, MD Professor, Program Director, Dermatology Residency, Department of

Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of

Dermatology, American College of Physicians, American Society of Nephrology, International
Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National
Kidney Foundation, Phi Beta Kappa, Women's Dermatologic Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for:
American Board of Dermatology<br/>Co-Editor for the text Dermatological Manifestations of
Kidney Disease .

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency

Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of

Dermatology, Society for Investigative Dermatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for:
nakedbiome<br/>Received income in an amount equal to or greater than $250 from:
elsevier;webMD<br/>editor in chief for: statpearls.

Additional Contributors

Craig A Elmets, MD Professor and Chair, Department of Dermatology, Director,

Chemoprevention Program Director, Comprehensive Cancer Center, UAB Skin Diseases
Research Center, University of Alabama at Birmingham School of Medicine

Craig A Elmets, MD is a member of the following medical societies: American Academy of

Dermatology, American Association of Immunologists, American College of Physicians,
American Federation for Medical Research, Society for Investigative Dermatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for:
University of Alabama at Birmingham; University of Alabama Health Services
Foundation<br/>Serve(d) as a speaker or a member of a speakers bureau for: Ferndale
Laboratories<br/>Received research grant from: NIH, Veterans Administration, California Grape
Assn<br/>Received consulting fee from Astellas for review panel membership; Received salary
from Massachusetts Medical Society for employment; Received salary from UpToDate for
employment. for: Astellas.

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