Subacute Cutaneous Lupus Erythematosus (SCLE) Clinical Presentation

  • Author: Jeffrey P Callen, MD; Chief Editor: William D James, MD   more...
 
Updated: May 15, 2012
 

History

Subacute cutaneous lupus erythematosus (SCLE) often begins as a papular eruption. Papules may show a photosensitive distribution. Many patients notice that sun exposure results in an exacerbation of their disease, and some report worsening each spring and summer. Patients may report mild pruritus, but most persons with SCLE are asymptomatic. Eventually, lesions develop into annular erythema or become psoriasiform in character. SCLE may wax and wane.

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 erythemaEarly lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption. Papulosquamous lesions of subacute cutaneous lupusPapulosquamous lesions of subacute cutaneous lupus erythematosus may simulate psoriasis. Annular lesions of subacute cutaneous lupus erytheAnnular lesions of subacute cutaneous lupus erythematosus.

Papulosquamous lesions may mimic psoriasis or lichen planus, while annular lesions may mimic erythema annulare centrifugum. Most patients exhibit 1 predominant type of lesion, and some also manifest isolated lesions of DLE.

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). In this author's opinion, PMLE and SCLE are distinctive disorders, but patients with recurrent photosensitive pruritic eruptions who are anti-Ro (SS-A) positive blur the distinction and might well be better classified as having SCLE rather than believed to have both disorders, as has been suggested by some investigators in Europe.

Unusual variants of SCLE

Tumid lupus erythematosus (TLE) involves a deeper, more nodular lesion in which little or no scaling is seen (see the image below). Some authorities have suggested that this variant is better classified as chronic cutaneous lupus erythematosus, while others have pointed out that this variant does not demonstrate an interface dermatitis upon histopathologic evaluation and therefore belongs in a separate subset among the skin lesions that are not histopathologically specific.[10]

Tumid lupus erythematosus. Tumid lupus erythematosus.

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

A variant including erythema multiforme–like lesions in association with DLE (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 also may have nonspecific cutaneous manifestations of lupus erythematosus (LE), such as livedo reticularis, palpable purpura, urticaria, ischemic changes of the distal fingertips (resulting from Raynaud phenomenon), or mucosal leukoplakic or ulcerative lesions.

Neonatal lupus erythematosus (NLE) most often manifests as a nonscarring form of LE (see the image below). Skin lesions are worsened by UV light and usually resolve by age 4-6 months. Some patients with NLE have congenital heart block. Patients with complete heart block eventually may require a pacemaker, may die suddenly, or may develop heart failure. NLE also may be manifested by cytopenias, and if thrombocytopenia is present, the neonate may have petechiae. Lastly, hepatosplenomegaly also may occur. Except for congenital heart block, all other manifestations resolve without intervention within 4-6 months.

Neonatal lupus erythematosus. Neonatal lupus erythematosus.
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Contributor Information and Disclosures
Author

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, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Celgene Honoraria Safety Monitoring Committee

Specialty Editor Board

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

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and 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, and 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 and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

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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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