Cutaneous Lupus Erythematosus

Cutaneous lupus erythematosus is an autoimmune disease that primarily targets the skin, causing various types of rashes and lesions. While some people experience only skin symptoms, others may develop signs of systemic disease affecting multiple organs. Understanding this condition and its triggers—especially sunlight exposure—is essential for managing symptoms and preventing complications.

Table of contents

• What Is Cutaneous Lupus Erythematosus?
• Medical Classification Codes
• Who Gets Cutaneous Lupus?
• What Causes Cutaneous Lupus?
• Types of Cutaneous Lupus
• Signs and Symptoms
• Diagnosis
• Treatment Options
• Living with Cutaneous Lupus
• Outlook and Prognosis

What Is Cutaneous Lupus Erythematosus?

Cutaneous lupus erythematosus is an autoimmune disease—a condition where the immune system mistakenly attacks the body’s own tissues. In this case, the immune system targets skin cells, causing chronic inflammation that shows up as various types of rashes and skin lesions^[1].

The term “cutaneous” means relating to the skin, so cutaneous lupus refers specifically to the skin symptoms of lupus. This condition is different from systemic lupus erythematosus (SLE), which affects the whole body and can cause inflammation in joints, blood vessels, and vital organs like the kidneys, heart, and lungs. While some people with cutaneous lupus only have skin problems, others may eventually develop systemic symptoms. In fact, cutaneous lupus can sometimes be the first sign of systemic lupus^[1].

Cutaneous lupus is actually two to three times more common than systemic lupus. Up to 75% of patients with systemic lupus develop skin signs at some point, and these skin symptoms may be the first indication of the disease in about 25% of cases^[3][7].

Medical Classification Codes

L93; L93.0; L93.1; L93.2; M32; M32.9

Who Gets Cutaneous Lupus?

Cutaneous lupus erythematosus has an annual incidence of 4 cases per 100,000 people and a prevalence of 73 cases per 100,000^[3]. Like systemic lupus, cutaneous lupus shows a marked female predominance, particularly affecting women between 20 and 50 years of age. However, all age groups and both sexes can be affected^[3].

Similar to systemic lupus, cutaneous lupus appears in approximately twice as many females as males. The highest peak of onset occurs in individuals in their third and fourth decades of life^[7].

Skin color is an important predisposing factor. While African Americans appear to be more likely to develop certain types of cutaneous lupus (specifically discoid lupus) than Caucasians, subacute cutaneous lupus patients show the opposite trend, with 85% of them being Caucasian^[7].

Although a specific genetic makeup can be a risk factor for developing cutaneous lupus, the vast majority of patients do not appear to have relatives with systemic lupus^[7].

What Causes Cutaneous Lupus?

The development of cutaneous lupus erythematosus is multifactorial, meaning several different factors work together to cause the disease^[3].

Genetic susceptibility plays an important role. There is a high incidence of the condition among family members. Various genes important in recognizing foreign proteins have been identified with higher frequency in cutaneous lupus patients. However, it is still unclear exactly how these genes lead to the formation of cutaneous lupus lesions^[7].

Environmental factors are significant triggers. Cigarette smoking and sun exposure are particularly important. Certain medications can also trigger cutaneous lupus symptoms^[3].

Sun exposure is an especially important activator of cutaneous lupus. Ultraviolet (UV) light radiation can cause skin manifestations by promoting cell death. These dead cells then become targets for autoantibodies—abnormal antibodies that react to the body’s own proteins and cause inflammation and damage. In addition, UV light radiation promotes the release of substances called cytokines, which activate and recruit inflammatory cells^[7].

The immune system itself is involved through innate and adaptive immune responses that produce autoantibodies. A skewed type I interferon production and response contribute to cutaneous lupus lesions^[3][9].

Types of Cutaneous Lupus

Cutaneous lupus can manifest in different ways, and healthcare providers classify it by how often symptoms occur and the types of skin lesions present. The condition is divided into several main categories^[1][3].

Acute Cutaneous Lupus Erythematosus (ACLE)

Acute cutaneous lupus is a lifelong condition, but the symptoms occur in an acute manner—they come on suddenly and then disappear for a while. It doesn’t tend to leave scars. This type typically presents as temporary red patches associated with a flare of systemic lupus erythematosus^[1][3].

The subtypes include localized ACLE (malar rash), generalized ACLE (maculopapular rash), and toxic epidermal necrolysis-like ACLE. The most recognizable sign is a painless malar rash or “butterfly rash”—redness and swelling over both cheeks and the bridge of the nose, sparing the folds beside the nose. This rash lasts hours to days^[3][7].

Half of systemic lupus patients will develop a malar rash, usually after sun exposure and often years before other symptoms appear^[15]. These lesions are sensitive to both sun rays and tanning rays and commonly appear on sun-exposed areas^[6].

Subacute Cutaneous Lupus Erythematosus (SCLE)

Subacute cutaneous lupus occurs for limited periods but repeatedly. For example, it may return every summer with sunlight exposure. It affects a deeper layer of skin below the surface^[1]. This type comprises 10-15% of cutaneous lupus presentations and is less commonly associated with systemic lupus, with approximately 50% having a mild form of systemic disease^[3].

The skin changes are more persistent than those of acute cutaneous lupus. Lesions occur on the trunk and upper limbs, triggered or aggravated by sun exposure. They present as either ring-shaped red patches with scaly borders and lighter centers, or as a raised rash that resembles psoriasis. The lesions resolve to leave pigmentation changes and tiny dilated blood vessels called telangiectases, but no scarring^[3][7].

It’s thought that 20-40% of subacute cutaneous lupus cases are drug-induced. Certain prescription drugs can cause this type, especially some heart medications, proton pump inhibitors, anti-fungals, chemotherapy medications, and tumor necrosis factor blockers^[3][15].

Intermittent Cutaneous Lupus Erythematosus

Intermittent cutaneous lupus is better known as lupus tumidus, a form of lupus that affects the deeper layer of skin. Skin lesions occur on sun-exposed areas such as the face, neck, and upper chest. They present as red, round or ring-shaped bumps and plaques with a smooth surface. These lesions resolve in winter without scarring^[3].

Chronic Cutaneous Lupus Erythematosus (CCLE)

Chronic cutaneous lupus causes steady skin symptoms that may get better or worse but never go away completely. The lesions it causes may leave permanent scars or skin discoloration^[1].

The most common form of chronic cutaneous lupus is discoid lupus erythematosus (DLE), named for its coin-shaped sores. These lesions are characterized by thickened, red scaly patches that often appear on the cheeks, nose, and ears. They may also appear on the v-shaped area of the neck, upper back, and back of the hands. Once the lesions heal, they may leave dark or light pigmentation as well as atrophy (thinning of the skin)^[6].

If lesions are in the scalp or involve hair follicles, areas of hair loss may develop, which could be permanent if the hair follicle is completely destroyed. Long-standing lesions may also predispose to future skin cancer. Individuals with discoid lupus on the head and neck do not usually develop systemic disease^[6].

Other subtypes of chronic cutaneous lupus include lupus profundus (also called lupus panniculitis), which affects fatty tissue below the skin resulting in firm deep nodules. Once these lesions resolve, they may leave indented scars due to destruction of fat cells. Chilblain lupus is another subtype^[1][6].

Signs and Symptoms

Cutaneous lupus causes several specific skin rashes and can also cause other skin symptoms that aren’t specific to lupus. Sometimes symptoms extend beyond the skin to the mouth, nostrils, and hair^[1].

Common symptoms include^[1]:

• Red, scaly areas of skin that might look like a coin or a ring, especially occurring on sun-exposed areas. Rashes might appear purple or brown on darker skin tones.
• A red rash that spreads across the cheeks and the bridge of the nose in the shape of a butterfly (malar rash).
• Patches of lighter and darker pigment on the skin, which are the remains of lesions that have healed.
• Photosensitivity—skin symptoms worsen with UV light exposure.
• Skin itching or irritation.
• Skin swelling, especially around the eyes.
• Hair loss related to scarring on the scalp.
• Mouth sores or sores inside the nostrils.

Other possible symptoms include hives, a mottled discoloration of the skin called livedo reticularis, Raynaud’s syndrome (where fingers and toes turn white or blue in response to cold or stress), spider veins, tiny purple spots called petechiae, and red palms^[1].

Individuals may also present with temporary hair loss, which is replaced by new hair once a disease flare is treated. Damage to blood vessels can result in small red-purple lesions on the lower legs called vasculitis^[6].

Diagnosis

Diagnosis of cutaneous lupus requires proper classification of the subtype through a combination of physical examination, laboratory studies, examination of tissue samples under a microscope, antibody tests, and occasionally a special test called direct immunofluorescence^[4].

During the physical examination, healthcare providers look at the characteristics of the rash, including its location, appearance, and whether it leaves scars. The pattern and type of skin lesions help determine which subtype of cutaneous lupus is present.

Laboratory studies may include blood tests to look for specific autoantibodies and markers of inflammation. A skin biopsy—removal of a small piece of skin for examination under a microscope—often shows characteristic changes. For cutaneous lupus, the tissue examination typically reveals vacuolar interface dermatitis, a specific pattern of inflammation where immune cells attack the layer between the outer skin and the deeper tissue^[3].

It is essential to ensure that systemic disease affecting other organs is excluded or identified. This may involve additional tests of kidney function, blood counts, and other organ systems^[4].

Treatment Options

There is no cure for cutaneous lupus. It is a chronic disease that can be controlled by a variety of medications and lifestyle changes^[7]. Treatment consists of patient education on proper sun protection along with appropriate topical and systemic medications^[4].

Sun Protection

Sun protection is a very important proactive measure to prevent skin lupus flares caused by UV radiation. Patients should be educated about the need for application and reapplication of sunscreen and the use of sun-protective clothing^[7][11].

Physical sunscreens (clothes, wide-brimmed hats, UV-filtering fabric) provide the best protection. Chemical sunscreens with a sun protection factor (SPF) of at least 30 and both UVA and UVB protection should be applied year-round to all exposed skin. People should stay out of the sun when it is strongest, typically between 10 AM and 4 PM^[6][7].

Topical Treatments

After appropriate sun protection measures, the initial treatment should include topical therapies. These include corticosteroids (medications that reduce inflammation), calcineurin inhibitors (another type of anti-inflammatory medication), and/or injections of glucocorticoids directly into the lesions^[11].

Topical tacrolimus 0.1% ointment has been shown to provide at least temporary benefit, especially in acute, swollen, non-thickened lesions^[11].

Systemic Medications

Systemic medications are indicated in cases of widespread, scarring, or treatment-resistant disease^[4].

Hydroxychloroquine is an antimalarial medication administered as first-line therapy to most patients with systemic disease. Research shows that antimalarials are 2.5 times more effective in lesions of acute cutaneous lupus compared with other cutaneous lupus skin lesion types. Patients with a more certain type of immune-driven disease may have more benefit from hydroxychloroquine^[9][11].

If disease persists despite treatment with hydroxychloroquine or topical therapies, doctors may recommend modification of antimalarial therapy and/or the addition of methotrexate, mycophenolate mofetil, anifrolumab, or belimumab^[11].

Systemic corticosteroid therapy for cutaneous lupus is suggested to be brief in duration because of potential adverse effects such as diabetes, high blood pressure, bone death, cataract development, and the risk of osteoporosis^[11].

Case reports continue to support the use of dapsone for cutaneous lupus, especially a blistering form called bullous lupus^[9].

Rituximab and belimumab have efficacy in patients with systemic lupus and severe active cutaneous lupus. Belimumab is a neutralizing antibody that inhibits the biologic activity of essential immune cells. It is approved for the treatment of adults and children with systemic lupus and has been shown to reduce autoantibody levels and help control disease activity^[9][11].

Anifrolumab, a human antibody to the type I interferon receptor, is approved for adults with moderate to severe systemic lupus. The significant role for type I interferons in cutaneous lupus and encouraging clinical data suggest anifrolumab as a very promising agent^[9][11].

Lenalidomide is conditionally recommended for ongoing moderate to severe cutaneous disease despite other measures^[11].

Other medications with supportive early data as promising new strategies for cutaneous lupus treatment include dapirolizumab, BIIB059, ustekinumab, and JAK inhibitors. The pipeline for new treatments is rich^[9].

Smoking Cessation

Advice to stop smoking is essential. Smoking is an environmental factor that can worsen cutaneous lupus^[11].

Living with Cutaneous Lupus

Living with cutaneous lupus can be challenging, but there are several strategies that can help manage the condition and improve quality of life.

Understanding your condition is powerful. Educating yourself about lupus, triggers for flares, and common treatments for symptoms helps you take charge of your care and better manage symptoms. In addition, educating others about your condition helps loved ones understand your daily struggles and better support you^[21].

Knowing your triggers helps avoid flares. For many with lupus, sun exposure can trigger a flare. Stress, exhaustion, infection, or even the period after childbirth can also trigger flares. When you know what triggers a flare, it becomes easier to help prevent them^[21].

Eating a heart-healthy diet that includes lots of fruits and vegetables, whole grains, and lean proteins is important. There is a strong link between lupus and heart disease, so eating well and exercising daily can help reduce risk. Make sure you get plenty of calcium and vitamin D, which can boost bone and immune system health^[21].

Moderate exercise when you’re feeling better can do your body good. It gets your joints moving to prevent stiffness, is good for maintaining a healthy weight and stress level, and can ward off fatigue. Exercise also can boost mood with the release of the body’s “feel-good” chemicals^[21].

Stress can have a considerable effect on the immune system. When people with lupus are under a lot of stress, their symptoms tend to worsen and it could lead to a flare. While stress is often unavoidable, you can learn to manage it more effectively with yoga and meditation, deep-breathing exercises, or talking with a therapist^[21].

Getting enough quality sleep is essential. Not getting enough sleep can increase inflammation in the body, which can worsen symptoms of pain, fatigue, and depression. Make sure you’re getting the recommended seven to nine hours of sleep each night to keep your energy up^[21].

Building a support network of people you trust can help you cope with lupus symptoms and flares. A therapist can also offer valuable support for overcoming both the emotional and physical challenges of living with lupus. There are many local and national support groups available as well^[21].

Outlook and Prognosis

Many people with lupus can minimize flares, lessen the burden of the disease, and live a quality life by following their treatment plan and taking care of themselves. In fact, nearly 90% of people with lupus live to a normal life expectancy^[21].

The outlook depends partly on which type of cutaneous lupus you have. Some types, like acute cutaneous lupus, don’t tend to leave scars, while chronic forms like discoid lupus may cause permanent scarring and skin discoloration^[1].

Resistance to conventional treatments is common, which can lead to an increased risk of scarring, disfigurement, and poor quality of life. This is why early diagnosis and appropriate ongoing treatment are so important^[9].

Some people with cutaneous lupus do go on to develop systemic symptoms. Sometimes cutaneous lupus is the first sign of systemic lupus. A certain percentage of people with subacute cutaneous lupus also develop systemic lupus or other autoimmune diseases. However, individuals with discoid lupus on the head and neck do not usually develop systemic disease^[1][6][15].

Continued research to understand the mechanisms driving cutaneous lupus will facilitate the development and approval of new treatments. With proper management and care, most people with cutaneous lupus can control their symptoms and maintain a good quality of life.