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Subacute Cutaneous Lupus Erythematosus (SCLE) Clinical Presentation

  • Author: Janice Lin, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Mar 07, 2016
 

History

Subacute cutaneous lupus erythematosus (SCLE) typically manifests in 1 of 2 forms: annular/polycyclic or psoriasiform/papulosquamous. SCLE lesions can begin as erythematous macules or papules that evolve into either annular polycyclic or psoriasiform plaques. Lesions typically occur in a photosensitive distribution. Many patients notice that sun exposure results in an exacerbation of their disease, and some report worsening each spring and summer. Photosensitivity is more frequently observed in patients with SCLE compared with discoid lupus erythematosus (DLE) lesions. Patients may report mild pruritus, but most persons with SCLE are asymptomatic.

Approximately 50% of patients with SCLE have accompanying joint involvement. Arthralgias are common, often symmetrical, and usually affect small joints, such as the wrists or those of the hands. Arthritis may occur but is unusual (< 2%).

Patients commonly complain of fatigue. Some patients have Sjögren syndrome, while others note dryness of their eyes and mouth. Patients may manifest symptoms of SLE; therefore, the history should include an assessment for symptoms of pleuritis, pericarditis, neurologic involvement, and renal impairment.

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Physical Examination

The primary lesion of subacute cutaneous lupus erythematosus (SCLE) is an erythematous papule or a small plaque with a slight scaling. Primary lesions expand and may merge and eventually form either plaques with scaling, in the papulosquamous variant, or annular and/or polycyclic lesions, in the annular variant. (See the images below.)

Early lesions of subacute cutaneous lupus erythema Early lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption.
Papulosquamous lesions of subacute cutaneous lupus Papulosquamous lesions of subacute cutaneous lupus erythematosus may simulate psoriasis.
Annular lesions of subacute cutaneous lupus erythe Annular lesions of subacute cutaneous lupus erythematosus.

Papulosquamous lesions may mimic psoriasis or lichen planus, while annular lesions may mimic erythema annulare centrifugum or tinea corporis. Most patients exhibit one predominant type of lesion, and some also manifest isolated lesions of discoid lupus erythematosus (DLE) during the course of their disease.

SCLE lesions primarily are photodistributed. When they occur on the lower extremities, they often are purpuric. Early lesions of SCLE may be difficult to distinguish from polymorphous light eruption (PMLE). Despite this, and the fact that both disorders have prominent photosensitivity, SCLE and PMLE are considered distinct disorders with differing histopathologic features.

Unusual variants of SCLE

Neonatal lupus erythematosus (NLE) most often manifests as a nonscarring form of lupus erythematosus (see the image below). Skin lesions are worsened by UV light and usually resolve by age 4-6 months. The cutaneous findings resemble those of SCLE. NLE lesions are typically annular erythematous plaques with a slight scale, which appear predominately on the scalp, neck, or face, but similar plaques may appear on the trunk or extremities. Infants with NLE should be evaluated for cardiac, hematologic, hepatic, and neurologic involvement. NLE is thought to be caused by the transplacental passage of maternal autoantibodies, most often anti-SSA/Ro, but also anti-SSB/La or anti-RNP antibodies.

Neonatal lupus erythematosus. Neonatal lupus erythematosus.

Annular erythema of Sjögren syndrome has been reported in Japanese and Polynesian patients. The authors believe that this is not a distinct entity, but rather SCLE with Sjögren syndrome in a particular ethnic population.

An infrequent variant including erythema multiforme–like lesions in association with lupus erythematosus (known as Rowell syndrome) and chilblains may exist, but it is not clear whether this is a distinct entity.

Patients with SCLE may have arthritis and pleuritis or pericarditis that manifest physical findings on joint or cardiopulmonary examination, respectively. Patients may also have nonspecific cutaneous manifestations of lupus erythematosus, such as livedo reticularis, palpable purpura, urticaria, ischemic changes of the distal fingertips (resulting from Raynaud phenomenon), or mucosal leukoplakic or ulcerative lesions.

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Contributor Information and Disclosures
Author

Janice Lin, MD, MPH Clinical Instructor, Department of Immunology and Rheumatology, Stanford University School of Medicine

Janice Lin, MD, MPH is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Medical Dermatology Society, Rheumatologic 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

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

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.

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Early lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption.
Papulosquamous lesions of subacute cutaneous lupus erythematosus may simulate psoriasis.
Annular lesions of subacute cutaneous lupus erythematosus.
Tumid lupus erythematosus.
Neonatal lupus erythematosus.
 
 
 
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