eMedicine Specialties > Dermatology > Connective Tissue Diseases

Lupus Erythematosus, Acute

Author: Charmaine Browne, MD, FRCPC, Department of Dermatology, Clinical Assistant Professor, University of Texas Health Science Center at San Antonio and University
Contributor Information and Disclosures

Updated: Nov 21, 2006

Introduction

Background

Lupus erythematosus (LE) is a heterogeneous connective-tissue disease associated with polyclonal B-cell activation and is believed to result from the interplay of genetic, environmental, and hormonal factors. The spectrum of disease involvement can vary from limited cutaneous involvement to devastating systemic disease.

From a dermatologic standpoint, the type of skin involvement can prove to be a good barometer of the pattern of underlying systemic activity. LE-specific skin diseases are recognized in 3 categories, including (1) acute cutaneous lupus erythematosus (ACLE), (2) subacute cutaneous lupus erythematosus (SCLE), and (3) chronic cutaneous lupus erythematosus (CCLE). Clinical characteristics of each group are unique, although histopathologically, only subtle differences are identified. The focus of this article is ACLE.

ACLE refers to a typical malar eruption in a butterfly pattern localized to the central portion of the face and/or a more generalized maculopapular eruption representing a photosensitive dermatitis. ACLE has a strong association with the systemic disease (see Image 4) for which patients present to rheumatologists and internists.

Pathophysiology

The etiology of LE is believed to be multifactorial, involving genetic, environmental, and hormonal factors. An association with human leukocyte antigen DR2 and human leukocyte antigen DR3 has been identified. Concordance in monozygotic twins and familial associations support a genetic basis in ACLE. In patients who are predisposed genetically, exposure to natural ultraviolet radiation is a frequent precipitating factor for LE.

Certain viruses (eg, Epstein-Barr virus, cytomegalovirus, HIV) have been implicated in precipitating or exacerbating LE.

Chemicals such as L-canavanine, which is present in alfalfa sprouts, have been known to induce systemic lupus erythematosus (SLE)–like illness. Drugs implicated in inducing an LE-like illness (eg, procainamide, isoniazid, hydralazine) are uncommonly associated with cutaneous manifestations.

Immunopathology

Data concerning direct immunofluorescence in ACLE are sparse. In one study, the results of 5 of 5 (100%) skin biopsies were reported as positive for the lupus band test. The lupus band test refers to the presence of immunoglobulins (Igs) and C3 complement components along the dermal-epidermal junction. All 3 immunoglobulin classes (immunoglobulin G [IgG], immunoglobulin M [IgM], immunoglobulin A [IgA]) and a variety of complement components have been identified at the dermal-epidermal junction. Recent research has shown that 60% of patients with a malar eruption of LE have positive lupus band test results. In nonlesional skin, positive lupus band test results correlate strongly with an aggressive course of systemic disease.

Frequency

United States

The malar rash has been reported in 20-60% of patients in large LE cohorts, while limited data suggest that the maculopapular eruption of SLE is present in 35% of patients with SLE.

Mortality/Morbidity

Significant morbidity and potential mortality are associated with SLE, of which ACLE is a manifestation.

Race

Precise data concerning the prevalence of ACLE in specific racial groups are not available; however, since photosensitivity is observed more frequently in whites than in blacks, the same prevalence for ACLE may be inferred. Estimates suggest that 1 in 250 black women in the US and the Caribbean and 1 in 1000 Chinese persons have SLE. Although LE may be rare in most parts of Africa, data concerning this finding conflict. Data concerning ACLE are difficult to interpret, since a lack of conformity is found in the description of lesions, and biopsy data are lacking for skin lesions observed in patients with systemic disease.

Age

The malar rash is believed to be associated with a younger age of disease onset.

Clinical

History

  • The most common presentation of ACLE is a red macular eruption involving the malar area. The forehead, periorbital area, and neck also may be involved, representing a photodistribution. Occasionally, unilateral involvement may occur (see Image 1).
  • Less commonly, ACLE presents as a generalized photosensitive eruption, while more rarely, patients present with widespread blistering simulating toxic epidermal necrolysis (TEN). TEN is believed to be a phototoxic reaction (see Image 2).
  • ACLE can be transient, lasting for several days to weeks. Lesions wax and wane with sun exposure over a period of several hours; however, some patients experience prolonged disease activity. Resolution of lesions may result in postinflammatory hyperpigmentation, especially in patients with darkly pigmented skin. Usually, the lesions are nonscarring.
  • Patients with ACLE frequently experience superficial ulceration of the oral and nasal mucosae. These lesions may produce extreme discomfort in some patients, although the lesions may be entirely painless in others. The posterior surface of the hard palate is the site affected most frequently; however, the gingival, buccal, and lingual mucosae also may be involved.
  • Note that ACLE may coexist with other LE-specific skin diseases. Localized ACLE lesions have been observed in 20% of SCLE patients; however, occurrence of ACLE with CCLE is unusual (see Image 3).

Physical

  • Primary lesions include the following:
    • Confluent erythema and edema
    • Erythematous macules and papules that eventually become confluent
    • Bullous lesions resembling TEN
    • Morbilliform macules and papules in a generalized photo-distributed pattern
  • Distribution of lesions
    • Malar eminence (representing the wings of the butterfly) and the nasal bridge (representing the body of the butterfly) typically are involved. Other sites of involvement include the forehead, periorbital area, and sides of the neck. Occasionally, a generalized photo-induced eruption may occur.
    • Associated findings include superficial ulceration primarily involving the posterior surface of the hard palate. Occasionally, buccal and gingival mucosae and the tongue may be involved.

Causes

In patients who are disposed genetically to developing SLE, the disease can be triggered by viruses (eg, EBV) and exposure to ultraviolet light. Medications typically do not induce ACLE in patients with drug-induced LE.

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References
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References

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Further Reading

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Keywords

butterfly rash, malar rash, photosensitive lupus dermatitis, acute lupus erythematosus, acute cutaneous lupus erythematosus, ACLE, LE, subacute cutaneous lupus erythematosus, SCLE, chronic cutaneous lupus erythematosus, CCLE

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Contributor Information and Disclosures

Author

Charmaine Browne, MD, FRCPC, Department of Dermatology, Clinical Assistant Professor, University of Texas Health Science Center at San Antonio and University
Charmaine Browne, MD, FRCPC is a member of the following medical societies: International Society of Dermatology, Royal College of Physicians and Surgeons of Canada, and Texas Medical Association
Disclosure: Nothing to disclose.

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

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

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

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: Nothing to disclose.

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

 
 
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