Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Discoid Lupus Erythematosus Clinical Presentation

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

History

Patients may report mild pruritus or occasional pain within the lesions, but most patients are asymptomatic. According to a recent epidemiologic study, approximately 16% of patients with discoid lupus erythematosus (DLE) may develop systemic involvement within 3 years of diagnosis.[2] Arthralgia or arthritis may occur.

Patients may manifest any sign or symptom of systemic lupus erythematosus (SLE); therefore, the history should include an assessment for serologic and/or hematologic abnormalities, arthritis, pleuritis, pericarditis, neurologic involvement, and renal involvement. A 2013 study demonstrated increased risk of photosensitivity, leukopenia, anti-Smith antibody, and decreased risk of arthritis and pleuritis in patients with SLE and DLE.[8] There was no significant association between DLE and anti-ds DNA antibodies, nephritis, or end-stage renal disease.

Malignant degeneration of chronic lesions of DLE leading to nonmelanoma skin cancer is rare. Dark-skinned individuals may be more prone to skin cancer because of the lack of pigmentation within the chronic lesion, combined with chronic inflammation and continued sun damage. Reported risk factors for the development of squamous cell carcinoma within lesions of chronic DLE include male sex, early age of onset, refractory disease, lip involvement, and tobacco use.[7]

Porphyria cutanea tarda (PCT) appears to be overrepresented in patients with cutaneous lupus erythematosus (CLE). Often, PCT is discovered when antimalarials are first administered. Lichen planus–like lesions may be due to an overlap between CLE and lichen planus or may occur as a result of antimalarial therapy.

Next

Physical Examination

Discoid lupus erythematosus (DLE) lesions frequently are characteristic. The primary lesion is an erythematous papule or plaque with slight-to-moderate scale (see the images below). As the lesion progresses, the scale may thicken and become adherent. Pigmentary changes may develop, with hypopigmentation in the central, or inactive area, and hyperpigmentation at the active border.

Discoid lupus erythematosus on the face. Discoid lupus erythematosus on the face.
Chronic scarred lesion of discoid lupus erythemato Chronic scarred lesion of discoid lupus erythematosus.

Lesions spread centrifugally and may merge. As lesions age, dilation of follicular openings occurs with a keratinous plug, termed follicular plugging or patulous follicles (see the image below). Resolution of the active lesion results in atrophy and scarring.

Lesions of discoid lupus erythematosus in the conc Lesions of discoid lupus erythematosus in the conchal bowl demonstrate patulous follicles with follicular plugging.

At any time, individual lesions may have any or all of these features. Early lesions may be difficult to distinguish from those of subacute cutaneous lupus erythematosus (SCLE). DLE lesions are often photodistributed, but relatively unexposed skin may also be affected. The conchal bowls and scalp are both common areas of involvement. Permanent alopecia may result (see images below).

Scarring alopecia of discoid lupus erythematosus. Scarring alopecia of discoid lupus erythematosus.
Widespread scarring alopecia. Widespread scarring alopecia.

Patients with DLE often are divided into 2 subsets: localized and widespread. Localized DLE occurs when the head and neck only are affected, while widespread DLE occurs when other areas are affected, regardless of whether disease of the head and neck is seen. Patients with widespread involvement often have hematologic and serologic abnormalities, are more likely to develop systemic lupus erythematosus (SLE), and are more difficult to treat.

Several unusual variants of chronic cutaneous lupus erythematosus (CCLE), other than DLE, have been reported. Mucosal surfaces may be affected by lesions that appear identical to DLE of the skin or by lesions that may simulate lichen planus. Palms and soles may be affected, but this occurs in less than 2% of patients (see the image below).[9]

Palmar lesions of discoid lupus erythematosus. Palmar lesions of discoid lupus erythematosus.

DLE lesions may become hypertrophic or verrucous (see the image below). This subset is manifested by wartlike lesions, most often on the extensor arms. Hypertrophic lesions of chronic lupus erythematosus must be differentiated from warts, keratoacanthomas, or squamous cell carcinoma. These lesions are more difficult to treat.[10]

Lupus panniculitis, a form of CCLE that manifests with inflammation and destruction of the subcutaneous fat, may be accompanied by overlying typical DLE lesions and/or may occur in patients with SLE.[11]

Hypertrophic lesions of chronic cutaneous lupus er Hypertrophic lesions of chronic cutaneous lupus erythematosus on the dorsal hands. Characteristic lesions were observed elsewhere.
Previous
 
 
Contributor Information and Disclosures
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)

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.

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.

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.

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: Nothing to disclose.

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.

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. Grönhagen 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, Hölzle 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].

  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].

  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, Brähler S, Bauer R, Bieber T, Tüting 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].

  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, Perusquía 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].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.