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Rare Cutaneous Manifestations of Systemic Lupus Erythematosus

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SLE is a chronic condition affecting many organs. Read the article below to learn more about its cutaneous manifestations.

Medically reviewed by

Dr. Dhepe Snehal Madhav

Published At April 18, 2024
Reviewed AtApril 23, 2024

Introduction:

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organs that can cause impairment and even death. One of its most common features is skin involvement. Gender is the biggest risk factor for SLE, with an incidence ratio of 3 to 4:1 in children and 7 to 15:1 in adults. The female-to-male ratio is still 3:1 in individuals with isolated cutaneous lesions despite a less pronounced gender preponderance in this group. It should be mentioned that among females aged 5 to 64, SLE ranks in the top 20 major causes of death. Black women are four times more likely than white women to have SLE, and patients of African heritage typically experience an earlier onset of the disease and a higher death rate. The article below briefs its rare cutaneous manifestations.

What Is Systemic Lupus Erythematous?

SLE, or systemic lupus erythematosus, is the most prevalent kind of lupus. An autoimmune condition called SLE causes extensive inflammation and tissue damage in the organs it affects due to the immune system attacking its own tissues. It may impact the blood vessels, brain, lungs, kidneys, joints, skin, and skin. Although there is no known cure for lupus, it can be managed with medication and a change in lifestyle. SLE is a multi-organ autoimmune disorder characterized by a variety of auto-antibody production patterns. Diseases range in severity from minor organ involvement (for example, cutaneous lesions) to severe organ involvement (for example, renal, nervous system, etc.) that can be fatal.

Causes:

The exact etiology of SLE is unknown. It might be connected to the following elements:

  • Environmental.

  • Genetic.

There are several known environmental SLE triggers. A number of medications have been linked to the demethylation of DNA (Deoxyribonucleic acid) and modification of self-antigens, which can result in a condition resembling lupus. More than 100 medications have been linked to drug-induced lupus, with Procainamide and Hydralazine having the highest prevalence.

UV radiation and sun exposure are well-known SLE triggers because they cause an increase in cell death. Molecular mimicry is believed to be the basic mechanism behind the several viral infections that have been linked to the issue. In comparison to the general population, children and adults with SLE had a higher prevalence of Epstein-Barr virus (EBV) antibodies. There is a dose response to the risk associated with smoking. Additional factors that may be dangerous include exposure to silica, other viral infections, insufficient vitamin D, alfalfa sprouts, and foods that contain canavanine.

Women are almost ten times as likely as men to have SLE. It might happen at any age. Yet young women between the ages of 15 and 44 seem to experience it the most frequently. African Americans, Asian Americans, African Caribbeans, and Hispanic Americans in the United States are more likely to contract the illness.

What Are the Complications of SLE?

SLE can impact a person's life in both the short and long term. The detrimental effects of SLE can be lessened, and the likelihood of improved function and quality of life can be increased with early diagnosis and efficient treatment. SLE can have more detrimental effects, leading to greater problems and a higher chance of death. These effects can be exacerbated by inadequate access to care, delayed diagnosis, ineffective medications, and poor adherence to treatment regimens.

A person's capacity for physical, mental, and social functioning may be limited by SLE. People with SLE may find their quality of life negatively impacted by these limits, particularly if they are fatigued. Fatigue is the most prevalent symptom that has a detrimental impact on an SLE patient's quality of life. Since employment is essential to a person's life, several studies utilize it as a metric to assess the quality of life for individuals with SLE. According to certain research, an individual's likelihood of working is inversely correlated with the length of time they have had SLE. Of those with SLE who are of working age, just 46 percent report having a job.

Treatment regimen adherence is frequently problematic, particularly for young women who are ready to have children (15 to 44 years old). In order to protect unborn children from injury, female patients with SLE must cease taking strong immunosuppressive drugs before and during pregnancy, as these medications can have major negative effects.

What Do Cutaneous Manifestations of SLE Mean?

After joint involvement, the skin is the most frequently affected organ, and skin lesions are the second most prevalent disease that manifests itself. More than 80 percent of SLE patients experience skin and mucous membrane involvement at some stage in their illness. About 45 percent of patients have some sort of vocational impairment as a result of their skin lesions, which cause significant morbidity through alopecia, scarring lesions, deformity, etc.

According to the Gilliam classification, skin lesions in SLE patients are divided into two categories: those of lupus non-specific diseases like alopecia (hair loss) and those of lupus-specific diseases like malar rash (butterfly rash). Due to environmental, cultural, and genetic variability, there is significant variance in the incidence, clinical heterogeneity, and severity of disease among various racial and ethnic groups.

Furthermore, whereas lupus non-specific skin lesions are linked to more active disease and so necessitate more severe therapy and disease surveillance, lupus-specific skin lesions primarily function as a crucial diagnostic tip. For effective diagnosis and treatment, a comprehensive understanding of cutaneous lesions in SLE is also essential. Even though cutaneous symptoms of SLE are prevalent, cutaneous lupus can develop without systemic lupus erythematosus. The three most prevalent forms of cutaneous lupus,

  • Erythematosus (CLE) is discoid lupus (DLE- round coin-shaped sores).

  • Subacute cutaneous lupus (SCLE- nonscarring photosensitive dermatosis).

  • Acute cutaneous lupus (ACLE- a butterfly-shaped rash across the nose and cheeks).

A number of less common cutaneous manifestations of lupus exist as well, such as lichenoid cutaneous lupus, lichen planus overlap syndrome, hypertrophic or verrucous discoid lupus and mucosal discoid lupus. The term "lupus" is also used to refer to several skin disorders that are not the same as lupus erythematosus, complicating matters: lupus vulgaris, lupus miliaris disseminatus faciei, and lupus pernio. Sarcoidosis, granulomatous rosacea, and tuberculosis, in that order, are associated with these skin problems.

Conclusion:

There is no known cure for lupus, a chronic inflammatory disease. If not properly managed, it can have a negative impact on numerous organs and result in a low standard of living. There are several reasons why people die too young. Educating and managing SLE patients by an interprofessional team can lower morbidity and mortality. Because SLE is a multisystem disorder, cutaneous symptoms can provide important information about the disease, both diagnostically (such as LE-specific skin lesions) and prognostically (such as LE non-specific skin lesions, which are linked to disease activity). Skin lesions cause higher morbidity. Therefore, a thorough grasp of SLE skin lesions will be beneficial for diagnosing the condition and effectively managing lupus patients. Consequently, increased collaboration between dermatologists and rheumatologists will result in more precise diagnosis of cutaneous lesions in SLE patients.

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Dr. Dhepe Snehal Madhav
Dr. Dhepe Snehal Madhav

Venereology

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