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Is Lupus Genetic? Cutaneous Lupus Description and Medication

Lupus is an inflammatory autoimmune disease. It affects the body and many different organ systems in a variety of different ways. Systemic lupus erythematosus (SLE) is the most common lupus diagnosis.

Lupus can cause inflammation and pain in any part of the body. It most commonly affects the skin, joints, and internal organs. During a lupus flare-up, the immune system attacks healthy tissue. That healthy tissue then becomes inflamed, leading to swelling and pain.

Lupus most commonly affects women. The Lupus Foundation of America reports that 9 out of 10 lupus patients are female, usually between the ages of 15 and 44. Lupus is diagnosed more often in people who are African American, Asian American, Hispanic/Latino, Native American, or Pacific Islander, and it is believed to have a genetic component.

About two-thirds of people with lupus develop a skin condition called cutaneous lupus erythematosus (CLE). This condition can cause rashes and sores all over the body. They most commonly appear on sun-exposed places like the face, ears, arms, neck, and legs. Exposure to ultraviolet light (whether from the sun or artificial light) can make these symptoms worse in 40–70 percent of people with lupus.

There are a few different types of cutaneous lupus. The three most common are: acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus.

People with acute cutaneous lupus erythematosus are typically fair-skinned women in their 30s. Acute CLE lesions occur when the systemic lupus response is active. 

Lesions from this type of lupus might be spread out all over the body or localized in one particular area (commonly above the neck). The most common localized lesion pattern is called a malar rash or butterfly erythema. This cutaneous lupus rash appears across the bridge of the nose and both cheeks, similar to a sunburn. The rash can last for anywhere from a few hours to a few days, but it does not normally leave any scarring. Generalized acute CLE (spread all over the body) is less common. When it does appear, it is often in areas with recent sun exposure.

Subacute cutaneous lupus erythematosus looks very different from acute CLE. It is more common among Caucasians. This rash looks like areas of red scaly skin with very clear edges or red ring-shaped lesions, typically on areas with the most sun exposure. The scalp and face are usually unaffected. Subacute CLE lesions don’t cause scarring but can change the skin pigmentation of affected areas after they heal.

About 15–20 percent of people with subacute CLE have other types of CLE lesions. Around 50 percent of people with subacute CLE also meet the criteria for SLE. Luckily, most do not develop a severe systemic disease.

Chronic cutaneous lupus is also known as discoid lupus. It is the most common subtype of CLE. About 60–80 percent of people with chronic cutaneous lupus have a localized rash above the neck. About 20–40 percent have a generalized cutaneous lupus rash spread over their body. Nearly all people with discoid lupus (70–90 percent) are photosensitive, and sun-exposed areas are most commonly affected.

Discoid lupus gets its name because of its presentation. This cutaneous lupus rash consists of disk-shaped, round lesions. The lesions are often red, scaly, and thick. They are usually not itchy or painful. When these lesions heal, they can cause scarring and pigmentation changes.

If the lesions occur on the head, they can cause hair loss. If the lesion leaves a scar after it heals, the hair loss may be permanent. Discoid lesions can also cause tissue loss and permanent change to skin texture on the ears and the tip of the nose.

Some less common forms of chronic CLE include lupus erythema (LE) hypertrophicus and LE profundus.

  • LE hypertrophicus — About 2 percent of people with CLE have this form of lupus. This condition rarely leads to systemic infection; however, its skin lesions can be chronic and may not respond to therapies.
  • LE profundus — This rare form of CLE shows up in the lower layers and fat tissue under the skin, typically in areas of increased fat deposits. It does not show up on the top layer of skin. Sun exposure usually doesn’t affect its growth. Treatment is important to prevent deep tissue scarring.

In addition to the different types of rash, cutaneous lupus has a few other symptoms.

  • Photosensitivity — Photosensitivity is when a person gets a rash after being exposed to ultraviolet light. Between 60 and 100 percent of people with lupus have photosensitivity issues. It is important to remember that other conditions can also cause photosensitivity.
  • Oral ulcers — About 12–45 percent of people with lupus experience ulcers. The condition does not have to be systemic to cause ulcers. Ulcers are also not specific to lupus.
  • Alopecia (hair loss) — Permanent scarring from discoid lupus can lead to hair loss. Temporary hair loss can also occur during a bad lupus flare-up. Telogen effluvium, which is hair thinning or shedding, can also happen when someone with lupus is very ill or has a reaction to their lupus medications.

Here are some other symptoms that are nonspecific to lupus:

  • Raynaud’s phenomenon — This condition causes a person’s fingers to turn white or blue when they are very cold.
  • Livedo reticularis — This is a net-like redness (with ulcerations) that usually occurs on the legs.
  • Vasculitis — Blood vessel inflammation can result from the disease itself, medication, or infection.

Lupus is a heterogeneous disease, meaning it looks and acts differently in different people. Researchers believe that lupus also has a genetic component that influences who gets the disease and its severity. Some people are born with lupus. Lupus can be caused by a genetic variation, where a person’s DNA causes their immune system to respond in inappropriate ways.

There are nearly 100 genetic variations known to affect lupus. Researchers are also studying why lupus is more common among women and why certain ethnic groups appear to be more susceptible. In the last five years, researchers have made an effort to include more non-European male subjects in lupus-related clinical trials.  

While heredity plays a large role in the development of lupus, more research is looking at the role of environmental triggers as well. This includes the use of certain drugs and medications, infections, and stress.

In cases where the skin is significantly damaged and unresponsive to other therapies, some people have tried treating cutaneous lupus lesions with phototherapy, photodynamic therapy, vascular laser therapy (for telangiectasia), and surgery (for bad disfigurations and scarring).

There is currently no medication that can cure lupus The goal of treatment for all forms of cutaneous lupus is to prevent flare-ups, prevent scars, and treat pigmentation changes. Medication for lupus depends on whether the person is receiving local therapy, systemic therapy, or severe disease therapy. Some form of steroid is usually the first-line lupus medication treatment, followed by or along with antimalarial drugs and/or immunosuppressants.

Local therapy

  • Topical steroids  are used for chronic discoid LE plaques.
  • Calcineurin inhibitors, pimecrolimus cream, or tacrolimus ointment can be used instead of steroids.
  • Intralesional corticosteroids are injected into small lesions when topical steroids are ineffective.
  • Makeup can be used to cover up plaques/lesions.

Systemic therapy

  • Antimalarial drugs — Over 70 percent of people with lupus respond to these medications. Hydroxychloroquine is the most commonly prescribed antimalarial drug for lupus.
  • Systemic corticosteroids
  • Vitamin D supplementation (with strict sun protection)
  • Immunosuppressants

Severe disease therapy

  • Immunosuppressants like cyclophosphamide
  • Thalidomide may be prescribed with extreme caution (can cause birth defects).
  • Photopheresis is when the person’s own blood is removed, separated, treated, and re-infused. 
  • Intravenous immunoglobulin is an infusion of plasma that provides antibodies and neutralizes autoantibodies.
  • An intravenous infusion of monoclonal antibodies targets T and B cells and cytokines.

Health care providers may prescribe immunosuppressants for lupus if steroids have been ineffective or the person can’t tolerate a high dose. These drugs can have serious side effects, including a decrease in the body’s ability to fight infection and an increased risk for certain types of cancer. Commonly prescribed medications include methotrexate, cyclophosphamide, and azathioprine.

When lupus is painful, your health care provider may prescribe you over-the-counter anti-inflammatories and pain relievers. For many people with lupus, these medications are enough to treat cutaneous lupus symptoms.

CLE can affect the body in many ways. Be patient as you and your health care provider work out a plan together. A successful cutaneous lupus erythematosus diagnosis and treatment plan usually starts with a physical exam, lab work, histology (microscopic exam of your cells), and blood work, so it may take some time. While CLE can be difficult to treat, there are a variety of care plans and medications that can successfully manage this chronic disease and its flare-ups.


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