What is Tumid Lupus Erythematosus?
Cutaneous lupus erythematosus (CLE), a skin disorder, can show up in three different forms: acute, subacute, and chronic. From these, the chronic version can itself have several subtypes such as tumid lupus erythematosus (TLE), discoid lupus erythematosus (DLE), chilblain lupus erythematosus, and lupus panniculitis. Even though these forms may appear different from one another, a patient can have more than one kind at the same time.
One subtype, named tumid lupus erythematosus (TLE), is unique from other forms of cutaneous lupus erythematosus because it rarely links with systemic lupus erythematosus (SLE), a more severe type of lupus that affects the whole body. This weak connection with SLE, and the lack of typical blood test abnormalities in TLE patients, leads some experts to think of TLE as a separate disease altogether. TLE has also been suggested to be related to other skin disorders, such as lymphocytic infiltrate of Jessner and reticular erythematous mucinosis (REM) due to similar changes found when studying skin tissue under a microscope.
Tumid lupus erythematosus (TLE) mostly appears as round, hard, red, swollen bumps and patches on the skin without any changes to the uppermost layer of skin. If these conditions are spotted, and changes to this upper layer of the skin are expected, discoid lupus erythematosus (DLE) might be considered as a diagnosis. TLE is commonly found on the face and trunk, and the skin lesions typically respond well to sun protection, topical corticosteroids (a type of anti-inflammatory medication), and antimalarials (medications used to prevent or treat malaria).
What Causes Tumid Lupus Erythematosus?
So far, doctors have not been able to identify a specific cause for TLE (Tubulointerstitial nephritis and uveitis). In some cases, things like sunlight exposure have been linked to making the condition worse. There’s also been some debate on whether TLE is related to autoimmune diseases, which are conditions where the body’s immune system attacks its own cells.
If a doctor suspects TLE is related to an autoimmune disease, they may perform what’s called an autoimmune workup. This is a series of tests to check for signs of autoimmune diseases.
One theory is that TLE could involve a disruption in the immune system that leads to the suppression of certain immune cells called T cells. T cells are important because they help your body fight off infections and diseases.
Few reports have also indicated a connection between TLE and habits or medications like smoking or certain drugs. These drugs include ones that lower the body’s immune response to diseases, ones that block substances which could harm the heart and blood vessels, certain medications that help get rid of extra fluid in the body by making you pee more, and special drugs used in treating HIV.
Risk Factors and Frequency for Tumid Lupus Erythematosus
CCLE, or chronic cutaneous lupus erythematosus, is more common in women. However, another type of lupus, TLE, or tumid lupus erythematosus, is quite rare and affects men and women equally. There is no clear data about how often TLE occurs in different races and ethnicities. Generally, TLE is first seen in people mainly aged thirty to forty.
Signs and Symptoms of Tumid Lupus Erythematosus
If you are seeking to diagnose Tumid Lupus Erythematosus (TLE), gathering detailed patient history can be quite informative. TLE might be possible if your skin condition worsens with exposure to the sun. However, this single symptom isn’t enough to confirm TLE. To make sure, a thorough skin exam covering the entire body is necessary when TLE is suspected. Pay extra attention to the face, neck, chest, and back, as TLE tends to affect these areas more. The skin lesions associated with TLE are usually swollen, ring-shaped, plaques that can vary in color from red to purple. Features of TLE skin lesions do not include atrophy, ulceration, follicular plugging, scarring or dyspigmentation. If any of these features are present, it may suggest a diagnosis of Discoid Lupus Erythematosus (DLE) instead.
More unique symptoms can sometimes be seen, such as a Blaschkoid distribution, swelling around the eyes, or scalp involvement that looks like alopecia areata. TLE skin lesions can persist for days or weeks and will often return over and over. While these can spontaneously disappear, recurrence is usually reported during the summer months.
Testing for Tumid Lupus Erythematosus
If the doctor believes you may have skin lupus, they will take a small sample of skin from a red, inflamed spot. This is called a biopsy. They might use a tool called a punch to remove the sample, which can be up to 4 mm on the body or 3 mm from sensitive spots like the face. The goal is to get a piece that goes through the full thickness of the skin. They will look for classic signs of lupus in the skin sample under a microscope, such as a large amount of mucin (a protein that makes up mucus) and lymphocytes (a type of white blood cell) surrounding blood vessels and adnexa, which are structures related to the skin like hair follicles.
If the results from looking at the skin sample under the microscope strongly suggest skin lupus is present, the doctor will then check if you have systemic disease, or lupus that affects multiple organs, even though there is a slim link between skin lupus and systemic lupus. To do this, the doctor will ask you about your health history and any symptoms you’re experiencing, perform a physical exam with a focus on swollen lymph nodes and joint inflammation, and order lab tests. These tests may include blood tests to look for specific antibodies that are often present with lupus, a urinalysis to examine your urine, a complete blood count to check on your overall blood health, and inflammatory marker tests such as ESR and CRP measurement. Sometimes, the doctor will run an extensive autoimmune screen, which involve drawing more blood to test for other autoimmune-related antibodies.
If your skin sample looks like it could be lupus but they aren’t completely sure, the sample could be tested using a method called direct immunofluorescence (DIF). This will look for deposits of certain types of antibodies (IgG and/or IgM) at the junction between the top and middle skin layer, as well as around the hair follicles. However, this test may or may not show anything helpful for diagnosing skin lupus, especially the tumid type of the disease.
Exposing your skin to light, a process called phototesting, may be useful for diagnosing skin lupus. If your skin develops lesions after exposure to UVA/UVB rays, it can support a diagnosis of the tumid form of skin lupus.
Treatment Options for Tumid Lupus Erythematosus
If you have localized TLE (a type of skin condition), doctors usually start treatment by using light protection and corticosteroids (medicines that reduce inflammation) applied to the skin or injected straight into the affected area. Sun protection is crucial and includes wearing sunblock with SPF 30 or higher, covering up with appropriate clothing, avoiding the sun at peak times, and stopping smoking.
Corticosteroid cream is typically applied twice a day for 2-4 weeks. Improvements are usually noticeable after about two weeks. Stronger corticosteroids are used for body and limb lesions, while milder ones are used for the face. There can be side effects like skin thinning, stretch marks, color changes, and spider veins. If your condition doesn’t improve after four weeks, your doctor may try different treatments.
In cases where the TLE lesions are isolated and thick, injections of a specific corticosteroid called triamcinolone into the lesion may be effective. Topical medicines known as calcineurin inhibitors can also help improve the lesion while reducing the use of corticosteroids. These do not cause skin thinning, making them ideal for long-term treatment.
If TLE doesn’t improve with topical treatments or if it’s widespread, antimalarial therapy is typically the next step. Hydroxychloroquine or chloroquine are usually the first choices. They’re generally well tolerated, but they do carry a risk of retinal toxicity (damage to the eye’s retina), so they should not be used together. Patients also need regular eye exams while on these medicines. Be aware that both drugs can cause changes to skin coloring—often a blue-grey color—that can be permanent. The most common reasons patients stop taking these medicines are gastrointestinal side effects, along with neuromuscular and blood abnormalities.
If TLE doesn’t respond to antimalarial therapy—which is rare—doctors may use second-line treatments like methotrexate or mycophenolate mofetil. Both these medications can have side effects like stomach upset and bone marrow suppression (when your body can’t make enough new blood cells), among others. Indeed, patients typically see improvement after 2-3 months of therapy.
Third-line treatments—used less commonly—include thalidomide and lenalidomide, both of which can cause several side effects, including birth defects if taken during pregnancy. These treatments are typically used for at least a month to see whether they’re effective, and then the doses are gradually reduced to maintain the improvements in the skin.
What else can Tumid Lupus Erythematosus be?
When diagnosing Tumid Lupus Erythematosus (TLE), doctors consider other conditions that might look similar:
- Jessner’s lymphocytic infiltrate: This presents similarly to TLE, with reddish bumps or nodules appearing without any flaky skin, usually on the upper back or the face. Lab tests may show a buildup of mucin, a type of protein, in the skin and positive results for certain antibody tests.
- Polymorphic light eruption (PMLE): This involves skin-colored or reddish bumps or patches that are itchy and typically appear on parts of the body exposed to the sun. These bumps show up within hours of sunlight exposure and disappear faster than TLE lesions. Unlike TLE, PMLE does not get worse with more exposure to UV light.
- Reticular erythematous mucinosis: This condition involves red overlay areas of the skin often appearing as a net-like pattern, typically on the upper back and chest. Lab tests would show more superficial mucin buildup and a more scattered and superficial presence of white blood cells called lymphocytes.
- Pseudolymphoma of the skin: This presents as red nodules in the skin, typically on the chest, arms, and face. These nodules are not sensitive to light. Lab tests would reveal the presence of certain cells, like histiocytes and plasma cells, along with lymphocytes.
- Granuloma faciale: This condition presents as a purplish bump or patch on the face, usually without any symptoms. The defining characteristic that distinguishes it from TLE is a thin zone of unaffected skin tissue that separates the inflamed area from the skin’s surface layer, known as the Grenz zone.
Moreover, it’s crucial for doctors to carefully review these possibilities and conduct specific tests to make an accurate diagnosis.
What to expect with Tumid Lupus Erythematosus
Tumid Lupus Erythematosus (TLE) often has a mild course. This means that solitary instances, or individual bumps or patches of skin affected by the condition, can sometimes go away on their own without the need for skin or body-wide treatment. However, it’s important to know that the condition often comes back, even though there may be periods where no symptoms are noticeable.
Some patients may experience long-term absence of symptoms. Skin issues related to TLE tend to have better outcomes compared to those from related conditions, such as Discoid Lupus Erythematosus or Subacute Cutaneous Lupus Erythematosus. This means that they can improve on their own without leaving permanent skin color changes or scars, sometimes in a matter of days or weeks. However, please remember that the condition might come back later on.
For over half of patients, protecting the skin from the sun alone was effective for getting rid of the skin problems without any other symptoms being present.
Possible Complications When Diagnosed with Tumid Lupus Erythematosus
It is infrequent for people to have both Systemic Lupus Erythematosus or discoid lupus erythematosus. Those with lupus often avoid the sun, making them potentially more likely to have a vitamin D deficiency. It’s recommended to check vitamin D levels, specifically the 25-hydroxyvitamin D level, and supplement with at least 400 IU of cholecalciferol if needed.
Noted Association:
- Infrequent link with Systemic Lupus Erythematosus or discoid lupus erythematosus
- Possible vitamin D deficiency due to avoidance of sun exposure
- Suggested check of 25-hydroxyvitamin D levels
- Recommended supplementation of at least 400 IU of cholecalciferol, if necessary
Preventing Tumid Lupus Erythematosus
Doctors strongly recommend patients to wear protective clothing and limit their exposure to the sun. Activities such as tanning, outdoor jobs, sunbathing, and traveling to areas near the equator should be avoided.
To further protect yourself from harmful sun rays, it’s advised to apply sunscreen at least twenty to thirty minutes before going out in the sun. The sunscreen should have a strength of at least 50 SPF (Sun Protection Factor), and should protect against both UVA and UVB types of radiation from the sun.
It’s also recommended that patients quit smoking, as it’s been reported to improve the condition of the affected skin area, particularly for those under antimalarial therapy, which is commonly used to treat this condition.
For those with more difficult cases, it is suggested to avoid using medications that increase sensitivity to light. Also, if a certain drug is causing this skin condition, stopping its use can be helpful. Patients should know that it’s normal for skin damages to appear up to three weeks after exposure to the sun.
Lastly, touching, scratching or applying pressure on the affected skin areas should be avoided. This is to prevent the Koebner phenomenon, a situation where skin diseases appear on areas of the skin that have been injured or traumatized.