eMedicine Specialties > Dermatology > Connective Tissue Diseases

Lupus Erythematosus, Subacute Cutaneous

Author: Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Apr 13, 2009

Introduction

Background

Subacute cutaneous lupus erythematosus (SCLE) is a nonscarring non–atrophy-producing photosensitive dermatosis. SCLE may occur in patients with systemic lupus erythematosus (SLE), Sjögren syndrome, and deficiency of the second component of complement (C2d), or it may be drug induced. Some patients also have the lesions of discoid lupus erythematosus (DLE), and some may develop small vessel vasculitis.

Patients with SCLE frequently fulfill 4 or more of the criteria used to classify SLE (see Systemic Lupus Erythematosus). Serologic abnormalities are common. Therapy with sunscreens, topical corticosteroids, and antimalarial agents is usually effective.

Pathophysiology

SCLE occurs in genetically predisposed individuals, most often in patients with human leukocyte antigen B8 (HLA-B8), human leukocyte antigen DR3 (HLA-DR3), human leukocyte antigen DRw52 (HLA-DRw52), and human leukocyte antigen DQ1 (HLA-DQ1). A strong association exists with anti-Ro (SS-A) autoantibodies. The reaction is believed to be related to ultraviolet (UV) light modulation of autoantigens, epidermal cytokines, and adhesion molecules, with resultant keratinocyte apoptosis.

Frequency

United States

Worldwide, SLE prevalence ranges from 17-48 cases per 100,000 persons. The highest prevalence of SLE occurs in patients aged 40-60 years. The male-to-female ratio of SLE is approximately 1:10. The male-to-female ratio of cutaneous lupus erythematosus (CLE) is approximately 1:2-3. Of patients with CLE, 10-50% have SCLE.

International

No differences in the prevalence of this disorder are recognized between the United States and other countries.

Mortality/Morbidity

Approximately one half of patients with SCLE have 4 or more of the criteria for classification as SLE, but in these patients, the disease is less severe, although in individual patients the full range of severity and end organ dysfunction is possible. By definition, skin lesions heal without scarring or atrophy but may leave residual dyspigmentation.

Race

SCLE is more common in whites (85%).

Sex

Male-to-female ratio of SCLE is 1:4.

Age

SCLE typically occurs in patients aged 15-70 years. The mean age is approximately 43 years.

Clinical

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 complain of mild pruritus, but most patients are asymptomatic.
    • Eventually, lesions develop into annular erythema or become psoriasiform in character.
  • SCLE may wax and wane.
  • Approximately 50% of SCLE patients have accompanying joint involvement.
    • Arthralgias are common, often symmetrical, and usually affect small joints such as hands and wrists.
    • 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.

Physical

  • The primary lesion of subacute cutaneous lupus erythematosus (SCLE) is an erythematous papule or a small plaque with a slight scaling (Media File 1). Primary lesions expand and may merge and eventually form either plaques with scaling (Media File 2) in the papulosquamous variant or annular and/or polycyclic lesions in the annular variant (Media File 3).
Early lesions of subacute cutaneous lupus erythem...

Early lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption.

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Early lesions of subacute cutaneous lupus erythem...

Early lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption.


Papulosquamous lesions of subacute cutaneous lupu...

Papulosquamous lesions of subacute cutaneous lupus erythematosus may simulate psoriasis.

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Papulosquamous lesions of subacute cutaneous lupu...

Papulosquamous lesions of subacute cutaneous lupus erythematosus may simulate psoriasis.


Annular lesions of subacute cutaneous lupus eryth...

Annular lesions of subacute cutaneous lupus erythematosus.

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Annular lesions of subacute cutaneous lupus eryth...

Annular 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 one 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.
  • Several unusual variants of SCLE have been reported.
    • Tumid lupus erythematosus (TLE) involves a deeper more nodular lesion in which little or no scaling is seen (Media File 4). 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 histopathological evaluation and therefore belongs in a separate subset among the skin lesions that are not histopathologically specific.
Tumid lupus erythematosus.

Tumid lupus erythematosus.

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Tumid lupus erythematosus.

Tumid lupus erythematosus.


    • Annular erythema of Sjögren syndrome has been reported in Japanese and Polynesian patients. The author believes 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 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 (Media File 5). 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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Neonatal lupus erythematosus.

Neonatal lupus erythematosus.


Causes

  • Patients are predisposed genetically to develop subacute cutaneous lupus erythematosus (SCLE).
  • Usually, the disease manifests following UV light exposure, but other triggers or inciting factors also must be implicated.
  • Exacerbation of disease or induction of lesions most commonly follows UV-B exposure. Some patients exhibit sensitivity to only UV-A or to both UV-A and UV-B.1
  • Several drugs may induce SCLE; the most frequently implicated is hydrochlorothiazide.2 Many pharmaceutical companies combine an antihypertensive agent with hydrochlorothiazide, and this should be assessed carefully. Additionally, implicated agents include calcium channel blockers, angiotensin-converting enzyme inhibitors, terbinafine, and tumor necrosis factor antagonists.3,4,5,6 Therefore, a careful drug history should be part of the initial evaluation of patients with SCLE.7
  • Patients with a deficiency of the second component of complement (C2d) often manifest SCLE lesions as part of the SLE-like disease.

More on Lupus Erythematosus, Subacute Cutaneous

Overview: Lupus Erythematosus, Subacute Cutaneous
Differential Diagnoses & Workup: Lupus Erythematosus, Subacute Cutaneous
Treatment & Medication: Lupus Erythematosus, Subacute Cutaneous
Follow-up: Lupus Erythematosus, Subacute Cutaneous
Multimedia: Lupus Erythematosus, Subacute Cutaneous
References
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References

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Further Reading

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Keywords

subacute cutaneous lupus erythematosus, SCLE, systemic lupus erythematosus, SLE, lupus, autoimmune disease, discoid lupus erythematosus, Sjogren syndrome

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Contributor Information and Disclosures

Author

Jeffrey P Callen, MD, Professor of Medicine, 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; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

Medical Editor

Kathleen David-Bajar, MD, Former Consultant to the Army Surgeon General, Department of Dermatology, Brooke Army Medical Center
Kathleen David-Bajar, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
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.

Managing Editor

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.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
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; american college of physicians Honoraria Other

 
 
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