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Systemic Lupus Erythematosus: Emerging Concepts: Part 2: Dermatologic and Joint Disease, the Antiphospholipid Antibody Syndrome, Pregnancy and Hormonal Therapy, Morbidity and Mortality, and Pathogenesis

Dimitrios T. Boumpas, MD; Barri J. Fessler, MD; Howard A. Austin III, MD; James E. Balow, MD; John H. Klippel, MD; and Michael D. Lockshin, MD
[+] Article, Author, and Disclosure Information

From the National Institutes of Health, Bethesda, Maryland. Request for Reprints: Dimitrios T. Boumpas, MD, National Institutes of Health, Building 10, Room 3N-112, Bethesda, MD 20892-1268. Acknowledgments: The authors thank Dr. Maria Turner for providing Figures 1, 2, and 3; Dr. Argyrios N. Theofilopoulos and Dr. Daniel L. Kastner for helpful discussions on the pathogenesis of systemic lupus erythematosus; and Ms. Lisa A. Miller and Mr. Andrew S. Lerner for preparation of the manuscript.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1995;123(1):42-53. doi:10.7326/0003-4819-123-1-199507010-00007
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Purpose: To review 1) advances in the pathogenesis, diagnosis, and management of dermatologic and joint disease and the antiphospholipid antibody syndrome in patients with systemic lupus erythematosus; 2) controversies related to pregnancy and hormonal therapy and to morbidity and mortality in these patients; and 3) current views on the pathogenesis of systemic lupus erythematosus.

Data Sources and Study Selection: Review of the English-language medical literature with emphasis on articles published within the last 5 years. More than 400 articles were reviewed.

Data Synthesis: Despite considerable overlap, cutaneous lesions specific to lupus erythematosus may be divided into subsets with distinct clinical, histologic, and immunofluorescent features. A recent short-term, prospective, uncontrolled trial found hydroxychloroquine and retinoids to be of similar efficacy in the treatment of cutaneous lupus erythematosus. Optimal treatment for patients with lupus and the anticardiolipin antibody syndrome remains to be defined; uncontrolled, retrospective, and treatment-withdrawal studies suggest that warfarin may be more protective than aspirin. Whether pregnancy induces lupus flares has not yet been established; existing data suggest both that it does and that it does not. Oral contraceptive use and postmenopausal estrogen replacement therapy appear not to cause clinical deterioration in patients with lupus. Recent studies have documented a substantial improvement in the survival of patients with systemic lupus erythematosus; they found 5-year survival rates of 90% or more and 10-year survival rates of more than 80%. Most data suggest that systemic lupus erythematosus results from the activation of self-reactive T cells and B cells by genetic or environmental factors.

Conclusions: The optimal treatment for dermatologic disease and the antiphospholipid antibody syndrome in patients with systemic lupus erythematosus remains unknown. Although mortality has decreased substantially, the morbidity related to the disease itself and to complications of therapy is still considerable. More studies are needed to further elucidate the effects of pregnancy on this condition and the pathogenetic mechanisms responsible for the development of this disease.


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Figure 1.
Cutaneous lupus erythematosus.Left.[1, 2]Right.

Localized acute cutaneous lupus erythematosus (malar dermatitis). These lesions are abrupt in onset, frequently appear after exposure to the sun, and are characterized by erythema and edema. The sparing of the nasolabial folds and the absence of discrete papules and pustules help to differentiate this condition from acne rosacea (including glucocorticoid-induced rosacea) . Other skin disorders, such as seborrheic or contact dermatitis, dermatophyte infections, and polymorphous light eruption may also be confused with malar dermatitis. Chronic cutaneous lupus erythematosus (discoid lupus erythematosus) with a malar distribution. Discoid lesions are usually found on the face, scalp, ears, or neck and begin as erythematosus papules or plaques with moderate scaling. As the lesion ages, the scale becomes thick and adherent and the follicular openings become dilated and filled with keratinous debris (follicular plugging). Eventually, pigmentary changes (hypopigmentation in the center and hyperpigmentation at the active border), atrophy, and scarring occur. (Courtesy of Dr. Maria Turner.).

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Figure 2.
Generalized acute cutaneous lupus erythematosus with areas of epidermal necrosis in a patient with systemic lupus erythematosus.[1]

These lesions developed abruptly after exposure to the sun. Other skin disorders, such as drug reaction, erythema multiforme, and toxic epidermal necrolysis, may result in similar rashes . (Courtesy of Dr. Maria Turner.).

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Figure 3.
Subacute cutaneous lupus erythematosus.leftright[1]

Typical features include symmetric, widespread, superficial, and nonscarring lesions. Involvement of the neck, shoulders, upper chest, upper back, and extensor surface of the hand is common. These lesions begin as small photosensitive, erythematous, scaly papules or plaques that evolve into a papulosquamous (psoriasiform) ( ) or annular polycyclic form ( ). Subacute cutaneous lupus erythematosus has been associated with the presence of anti-Ro/SS-A antibodies, genetic deficiencies of complement C2 and C4, and certain medications, such as hydrochlorothiazide . (Courtesy of Dr. Maria Turner.).

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Figure 4.
Jaccoud arthropathy in a patient with systemic lupus erythematosus.topmiddlebottom

Deformities in the hands ( ), such as ulnar drift at the metacarpophalangeal joints, swan-neck and boutonniere deformities, and hyperextension at the interphalangeal joint of the thumb closely resemble those seen in rheumatoid arthritis. The absence of erosions on radiographs ( ) and their reducibility ( ) are helpful in distinguishing this condition from the deforming arthritis of rheumatoid arthritis.

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