Approach Considerations

Screening for systemic lupus erythematosus (SLE) should occur upon diagnosis of discoid lupus erythematosus (DLE). This should consist of a thorough history and physical examination, as well as standard laboratory screening including complete blood cell count, renal function tests, and urinalysis. Hematologic and serologic abnormalities may be present and an elevated sedimentation rate may occur in some patients. Additionally, rheumatoid factor may be positive and complement levels may be decreased. Abnormal renal function tests and/or urinalysis with proteinuria may reflect the presence of renal involvement.

Some patients with DLE (approximately 20%) manifest a positive antinuclear antibody (ANA) when tested with human substrates. HEp-2 cells currently are the most common substrate used in commercial laboratories.

Anti-Ro (SS-A) autoantibodies are present in up to 20% of patients. Antinative deoxyribonucleic acid (DNA, either double-stranded or nDNA) or anti-Sm antibodies usually reflect SLE, and they may occur in some patients (5-20%).^[25]In one study, levels of ANA, anti-RNP, anti-dsDNA, and anti-ssDNA IgG were associated with disease activity of DLE as determined by the Cutaneous Lupus Disease Area and Severity Index (CLASI).^[26]

Other Tests

Deposition of immunoglobulin and/or complement at the dermoepidermal junction is a characteristic feature of lupus erythematosus. Tissue may be examined from skin lesions (lesional) or normal skin (nonlesional). Nonlesional biopsies may be from photoexposed or nonexposed surfaces. Testing of nonlesional, nonexposed skin is termed the lupus band test (LBT).

The use and interpretation of these tests vary according to the biopsy site. Approximately 90% of patients with discoid lupus erythematosus (DLE) manifest a positive direct immunofluorescence (DIF) test on lesional skin; however, the presence of immunoreactants in the basement membrane zone of lesional skin is not specific for lupus and can be seen in a variety of inflammatory skin diseases. Older lesions or very early lesions may be more likely to be negative on immunofluorescence microscopy. Most commonly, DLE may be diagnosed by clinical examination and histopathologic findings, rather than relying on DIF testing.

Only patients with systemic lupus erythematosus (SLE) have a positive LBT, defined as the presence of multiple immunoreactants in the basement membrane zone. The LBT is neither sensitive nor specific and has mostly been replaced by advances in serologic testing.

Histologic Findings

The characteristic histopathologic alterations observed in discoid lupus erythematosus (DLE) include the following:

Vacuolar alteration of the basal cell layer
Thickening of the basement membrane
Follicular plugging
Hyperkeratosis
Atrophy of the epidermis
Incontinence of pigment
Inflammatory cell infiltrate (usually lymphocytic) in a perivascular, periappendageal, and subepidermal location

Often, an abundance of mucin is seen within the dermis. The histopathologic features differ depending on the type and age of the lesion.

Treatment & Management

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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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Author

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.

Coauthor(s)

Sheena Desai MD Candidate, Tufts University School of Medicine; MBA Candidate, Brandeis University, The Heller School for Social Policy and Management

Sheena Desai is a member of the following medical societies: American Medical Association, Massachusetts Medical Society, Medical Dermatology Society, Skin of Color Society

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: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) Received honoraria from UpToDate for author/editor; 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 these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Specialty Editor Board

David F Butler, MD Former 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: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Julia R Nunley, MD Professor, 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 Author for: Up-to-date.

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: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD Served as a speaker for various universities, dermatology societies, and dermatology departments.

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 Serve(d) as a speaker or a member of a speakers bureau for: Ferndale Laboratories Received research grant from: NIH, Veterans Administration, California Grape Assn 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.

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.

Elizabeth Tkachenko University of Massachusetts Medical School

Disclosure: Nothing to disclose.