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Lupus Erythematosus, Acute
Updated: Jul 15, 2009
Introduction
Background
Lupus erythematosus 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. Lupus erythematosus–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 acute cutaneous lupus erythematosus.
Acute cutaneous lupus erythematosus 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. Acute cutaneous lupus erythematosus has a strong association with the systemic disease (see Media File 4) for which patients present to rheumatologists and internists.
Relationship of acute cutaneous lupus erythematosus (ACLE) to systemic disease. Lupus erythematosus is lupus erythematosus. CCLE is chronic cutaneous lupus erythematosus. SCLE is subacute cutaneous lupus erythematosus.
Pathophysiology
The etiology of lupus erythematosus 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 acute cutaneous lupus erythematosus.
More than 25 genes have been identified as contributing to the mechanisms that predispose patients to lupus. They include alleles in the major histocompatibility complex region (multiple genes): IRF5, ITGAM, STAT4, BLK, BANK1, PDCD1, PTPN22, TNFSF4, TNFA1P3, SPP1, some fc gene receptors, and deficiency in several complement components, including C1qC4+C2. In patients who are predisposed genetically, exposure to natural ultraviolet radiation is a frequent precipitating factor for lupus erythematosus.1,2
Certain viruses (eg, Epstein-Barr virus, cytomegalovirus, HIV) have been implicated in precipitating or exacerbating lupus erythematosus.
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 a lupus erythematosus–like illness (eg, procainamide, isoniazid, hydralazine) are uncommonly associated with cutaneous manifestations.
Also see Lupus Erythematosus, Bullous; Lupus Erythematosus, Discoid; Lupus Erythematosus, Drug-Induced; and Lupus Erythematosus, Subacute Cutaneous.
Immunopathology
Data concerning direct immunofluorescence in acute cutaneous lupus erythematosus are sparse. In one study, the results of 5 (100%) of 5 skin biopsy specimens were reported as positive for the lupus band test. The lupus band test refers to the presence of immunoglobulins and C3 complement components along the dermoepidermal 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 dermoepidermal junction. Research has shown that 60% of patients with a malar eruption of lupus erythematosus have positive lupus band test results. In nonlesional skin, positive lupus band test results correlate strongly with an aggressive course of systemic disease.3Frequency
United States
The malar rash has been reported in 20-60% of patients in large lupus erythematosus cohorts, while limited data suggest that the maculopapular eruption of systemic lupus erythematosus is present in 35% of patients with systemic lupus erythematosus.
Mortality/Morbidity
Significant morbidity and potential mortality are associated with systemic lupus erythematosus, of which acute cutaneous lupus erythematosus is a manifestation.
Race
Precise data concerning the prevalence of acute cutaneous lupus erythematosus in specific racial groups are not available; however, since photosensitivity is observed more frequently in whites than in blacks, the same prevalence for acute cutaneous lupus erythematosus may be inferred. Estimates suggest that 1 in 250 black women in the US and the Caribbean and 1 in 1000 Chinese persons have systemic lupus erythematosus. Although lupus erythematosus may be rare in most parts of Africa, data concerning this finding conflict. Data concerning acute cutaneous lupus erythematosus 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 acute cutaneous lupus erythematosus 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 Media File 1).
Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
- Less commonly, acute cutaneous lupus erythematosus 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 Media File 2).
Toxic epidermal necrolysis–like eruption.
- Acute cutaneous lupus erythematosus 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 acute cutaneous lupus erythematosus 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 acute cutaneous lupus erythematosus may coexist with other lupus erythematosus–specific skin diseases. Localized acute cutaneous lupus erythematosus lesions have been observed in 20% of subacute cutaneous lupus erythematosus patients; however, occurrence of acute cutaneous lupus erythematosus with chronic cutaneous lupus erythematosus is unusual (see Media File 3).
Relationship of acute cutaneous lupus erythematosus (ACLE) to chronic cutaneous lupus erythematosus (CCLE) and subacute cutaneous lupus erythematosus (SCLE).
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 systemic lupus erythematosus, the disease can be triggered by viruses (eg, EBV) and exposure to ultraviolet light. Medications typically do not induce acute cutaneous lupus erythematosus in patients with drug-induced lupus erythematosus.
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References
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Further Reading
Keywords
acute lupus erythematosus, acute cutaneous lupus erythematosus, ACLE, LE, subacute cutaneous lupus erythematosus, SCLE, chronic cutaneous lupus erythematosus, CCLE, butterfly rash, malar rash, photosensitive lupus dermatitis
