Chronic Cutaneous Lupus Erythematosus
Chronic cutaneous lupus erythematosus is a form of autoimmune disease that primarily affects the skin, causing persistent rashes and lesions that may lead to permanent scarring and changes in skin color. Unlike lupus that affects the whole body, this condition focuses on the skin, though it can sometimes be an early warning sign of more widespread disease.
Table of contents
- What is Chronic Cutaneous Lupus Erythematosus?
- Types of Chronic Cutaneous Lupus
- Who Gets This Condition?
- What Causes Chronic Cutaneous Lupus?
- Signs and Symptoms
- How Doctors Diagnose This Condition
- Treatment Options
- Self-Care and Lifestyle Changes
- Living With Chronic Cutaneous Lupus
What is Chronic Cutaneous Lupus Erythematosus?
Chronic cutaneous lupus erythematosus (CCLE) is one type of cutaneous lupus, which means skin lupus. It is an autoimmune disease, a condition where your immune system mistakenly attacks your own body tissues instead of protecting you from harmful invaders[1]. In chronic cutaneous lupus, the immune system attacks skin cells, causing ongoing inflammation that shows up as various skin problems[2].
The word “chronic” means that the skin symptoms are steady and long-lasting. They may get better or worse over time, but they never completely disappear[1]. This is different from other forms of cutaneous lupus where symptoms come and go suddenly or return only during certain times of the year.
Chronic cutaneous lupus is two to three times more common than systemic lupus erythematosus (SLE), which is the form of lupus that affects the whole body[4]. While most people with chronic cutaneous lupus have only skin problems, about 5 to 20 percent may eventually develop systemic lupus[5][7]. For some patients, chronic cutaneous lupus can be the first sign of more widespread disease[1].
Types of Chronic Cutaneous Lupus
There are several subtypes of chronic cutaneous lupus, each with its own characteristics:
Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus[5]. It is named for the disc-shaped or coin-shaped rashes it creates. These lesions are typically red, scaly, and raised patches that appear most often on the face, scalp, and ears[5][6]. The rash can also spread to the neck, upper back, and backs of the hands.
The lesions in discoid lupus are often not itchy or painful[5]. However, when they heal, they leave behind areas of darker or lighter skin color and thinning of the skin[5]. When discoid lupus affects the scalp or hair follicles, it can cause permanent hair loss if the hair follicle is completely destroyed[5]. People with discoid lupus limited to the head and neck area usually do not develop systemic disease[5].
Lupus profundus, also called lupus panniculitis, affects the deeper fatty tissue below the skin[1][5]. This type creates firm, deep nodules under the skin. When these lesions heal, they may leave indented scars due to the destruction of fat cells[5].
Chilblain lupus is another subtype of chronic cutaneous lupus that appears in areas exposed to cold[1][2].
Lupus tumidus, sometimes called intermittent cutaneous lupus, presents as smooth, raised red nodules or ring-shaped patches on sun-exposed areas like the face, neck, and upper chest[2]. These lesions typically resolve without scarring and may improve during winter months[2].
Who Gets This Condition?
Chronic cutaneous lupus affects certain groups of people more than others. Women are affected about two to three times more often than men[2][17]. The condition most commonly appears in people between their 20s and 50s, with the highest number of new cases occurring in people in their 30s and 40s[2][4].
Skin color plays an important role in who develops chronic cutaneous lupus. People with darker skin tones are more likely to develop this condition[2]. Discoid lupus, the most common subtype, is more frequent in African Americans than in people of European descent[4]. However, subacute cutaneous lupus, which is a different form of skin lupus, shows the opposite pattern, with about 85 percent of patients being of European descent[4].
While having a family member with lupus can increase risk, most people with cutaneous lupus do not have relatives with systemic lupus[17].
What Causes Chronic Cutaneous Lupus?
The exact cause of chronic cutaneous lupus is not fully understood, but researchers believe several factors work together to trigger the disease[4][17].
Genetic factors appear to play a role. Certain genes that help the body recognize foreign substances have been found more frequently in people with cutaneous lupus[17]. There is also a higher incidence of lupus among family members, suggesting that genetic susceptibility is important[2].
Environmental triggers can activate the disease in people who are genetically predisposed. Sunlight exposure is one of the most important triggers[1][17]. Ultraviolet light radiation can cause skin cells to die, and these dead cells become targets for autoantibodies, which are abnormal antibodies that attack the body’s own proteins[17]. Ultraviolet light also promotes the release of substances called cytokines that activate and recruit inflammatory cells[17]. Other environmental factors include cigarette smoking, certain medications, and viral infections[2][4].
Immune system abnormalities are central to the development of chronic cutaneous lupus. The body produces an excessive amount of type I interferons, which are proteins that normally help fight infections[11]. In cutaneous lupus, this interferon response is skewed and contributes to inflammation in the skin[11].
Skin lupus, CCLE, Discoid lupus, DLE, Lupus panniculitis, Lupus profundus, Chilblain lupus, Lupus tumidus
L93, L93.0, L93.1, L93.2
Signs and Symptoms
The symptoms of chronic cutaneous lupus primarily affect the skin, but they can extend to other areas like the hair, mouth, and nostrils.
The most common symptom is red, scaly patches on the skin[1]. These areas might look like coins or rings and appear especially on parts of the body exposed to sunlight, such as the face, ears, scalp, neck, and arms[1][5]. On darker skin tones, these rashes might appear purple or brown instead of red[1].
Skin discoloration is another common feature. After lesions heal, they often leave behind patches of lighter or darker skin pigment[1]. The skin may also become thinner in affected areas[5].
Photosensitivity, meaning that skin symptoms get worse with exposure to ultraviolet light, is a hallmark of chronic cutaneous lupus[1]. Both natural sunlight and artificial sources like tanning beds can trigger or worsen symptoms.
Some people experience itching or irritation of the skin, though this is not always present[1][6]. Swelling, especially around the eyes, can also occur[1].
Hair loss is a significant concern when chronic cutaneous lupus affects the scalp[1][5]. The scarring that results from healed lesions can permanently destroy hair follicles, leading to permanent bald patches.
Mouth sores or sores inside the nostrils may develop in some people[1]. When lesions affect the inside of the mouth or lips, they can cause ulcers and carry a risk of developing into skin cancer in the future[5].
Long-standing lesions may also predispose to the development of skin cancer[5]. Always wearing sunscreen on the rash and wearing a wide-brimmed hat greatly decrease this risk[6].
- Skin
- Scalp
- Face
- Ears
- Neck
- Hair follicles
- Mouth
- Nostrils
How Doctors Diagnose This Condition
Diagnosing chronic cutaneous lupus requires several steps because there is no single specific test for the condition[8]. Doctors use a combination of approaches to properly classify the subtype and rule out systemic disease[4].
The process begins with a complete physical examination and a detailed discussion about your symptoms, medical history, and family health history[8]. Your doctor will carefully examine your skin lesions, noting their appearance, location, and characteristics.
Blood tests play an important role in diagnosis[8]. Common tests include the ANA (antinuclear antibody) test, complete blood count, and complement tests[8]. These tests help determine whether there is systemic involvement and can identify specific antibodies associated with lupus.
A skin biopsy is usually necessary to confirm the diagnosis[4][7]. During this procedure, a small sample of affected skin is removed and examined under a microscope. The biopsy shows characteristic changes called vacuolar interface dermatitis, which means there is specific inflammation at the junction between different layers of skin[2].
Direct immunofluorescence testing may occasionally be performed on the skin biopsy sample[4]. This special test can detect immune deposits in the skin.
Your doctor may also order urine tests to check for kidney involvement[8], since lupus can sometimes affect the kidneys even when skin symptoms are the main concern.
Treatment Options
While there is no cure for chronic cutaneous lupus, various treatments can control symptoms and prevent further damage[17]. Treatment goals include improving existing lesions, preventing new lesions from forming, and reducing associated symptoms[16].
Sun Protection
Sun protection is the foundation of treatment for chronic cutaneous lupus[16][17]. Broad-spectrum sunscreen with a sun protection factor (SPF) of 50 or greater should be applied daily, at least 20 minutes before sun exposure, and reapplied every 2 to 3 hours[16]. Sun avoidance and sun-protective clothing, including wide-brimmed hats, are also necessary[16].
Topical Treatments
Topical corticosteroids are often the first medication used for limited disease[11][14]. These creams or ointments reduce inflammation directly on the skin. Calcineurin inhibitors are another type of topical medication that can be used to control inflammation[14].
Antimalarial Medications
Antimalarial therapy is considered the gold standard of systemic treatment for chronic cutaneous lupus[4][11]. These medications, which include hydroxychloroquine and chloroquine, were originally developed to treat malaria but have proven effective for skin lupus. In patients whose skin lesions do not respond well to hydroxychloroquine alone, adding quinacrine can improve results[16].
Other Systemic Medications
For more widespread, scarring, or treatment-resistant disease, systemic medications may be necessary[4]. Options include:
- Systemic corticosteroids can help control inflammation throughout the body[14].
- Immunosuppressants like methotrexate and mycophenolate mofetil work by dampening the overactive immune response[1].
- Retinoids (vitamin A derivatives) may be used in some cases[4].
- Dapsone has shown benefit in case reports, especially for certain forms of lupus[4].
Biologic Therapies
Rituximab and belimumab are monoclonal antibody medications that have shown efficacy in patients with systemic lupus and severe active cutaneous lupus[1][4]. These medications target specific parts of the immune system.
Anifrolumab, which blocks type I interferon receptors, is a very promising agent for cutaneous lupus based on encouraging clinical data[11]. Other emerging treatments include JAK inhibitors, which target specific enzymes involved in inflammation[11].
Other Therapies
Additional treatment options that may be considered include gold compounds, immunomodulators, and in some cases, pulsed dye laser therapy[14].
Self-Care and Lifestyle Changes
Living with chronic cutaneous lupus requires active participation in your own care. Several self-care measures can help you manage the condition and prevent flares.
Strict sun protection cannot be overemphasized. This means not only wearing sunscreen daily but also seeking shade, wearing protective clothing, and avoiding peak sun hours[17][20]. Physical sunscreens that contain ingredients like zinc oxide or titanium dioxide provide a barrier against ultraviolet rays[17].
Smoking cessation is crucial. Cigarette smoking is an environmental factor that can trigger or worsen cutaneous lupus[2]. If you smoke, quitting should be a priority.
Medication adherence is one of the most important aspects of managing chronic cutaneous lupus. Taking medicines exactly as prescribed by your doctor helps keep the disease under control[19]. If you think you are not tolerating your treatments well, let your doctor know immediately[19].
Regular doctor visits and lab work are essential even when you feel well[19]. Lupus can become active in ways that only laboratory tests can detect. Research confirms that people with lupus who see their specialists regularly have better health outcomes[19].
Gentle skin care is important. Use mild, fragrance-free cleansers and moisturizers. Avoid products that irritate your skin or trigger symptoms.
Learning about your condition helps you feel more in control[19]. The more you know about what triggers flares for you, the better you can avoid those triggers and stay symptom-free[19].
Communication with your healthcare team is vital. Be open about all symptoms you experience and any changes in your condition[19]. Knowing what you were doing or experiencing when a flare developed helps your doctor help you better.
Living With Chronic Cutaneous Lupus
Chronic cutaneous lupus is a lifelong condition that requires ongoing management[17]. The disease can vary significantly from person to person in terms of severity and progression.
Many people with chronic cutaneous lupus limited to the skin do not develop systemic disease. However, it is important to monitor for signs of systemic involvement over time, as skin symptoms can sometimes be the first indication of broader disease[1].
The emotional and psychological impact of chronic cutaneous lupus should not be underestimated. Visible skin changes, scarring, and hair loss can affect self-esteem and quality of life. Seeking support from family, friends, or support groups can be helpful. Many organizations offer resources and connections with others who understand what you are experiencing.
The outlook for people with chronic cutaneous lupus has improved with advances in treatment. While the condition cannot be cured, many people achieve good control of their symptoms with appropriate therapy and lifestyle modifications. The risk of permanent scarring and disfigurement can be reduced when treatment is started early and sun protection is practiced consistently.
Research into the mechanisms driving chronic cutaneous lupus continues to advance[11]. The pipeline for new treatments is promising, with several novel therapies showing encouraging results in clinical trials[11]. As our understanding of the disease improves, more targeted and effective treatments are becoming available.
Working closely with a dermatologist and, when needed, a rheumatologist is essential for optimal disease management[6]. These specialists can help prevent permanent scarring, hair loss, and other complications while addressing your individual concerns and treatment goals.
