Lupus erythematosus (butterfly lichen, butterfly erythema) is an autoimmune disease that mainly occurs in women. The immune system mistakenly attacks the body’s cellular structures. Most of the time, the disease is more or less limited to the skin, as in cutaneous lupus erythematosus. Internal organs (systemic lupus erythematosus) can also be affected. Read more about the causes, symptoms, diagnosis, and treatment of lupus.
What is lupus erythematosus?
A rare chronic inflammatory autoimmune disease that mainly affects young women.
Two main forms: cutaneous lupus erythematosus (CLE) and systemic lupus erythematosus (SLE).
Symptoms: CLE affects the skin only with typical butterfly-shaped skin changes on parts of the body exposed to the sun. SLE also affects internal organs (e.g., inflammation of the kidneys, joint pain).
How dangerous is the disease? Lupus has a good prognosis (even if it is incurable). Sometimes it turns into systemic lupus. Life expectancy is then also normal.
Causes and risk factors: The probable cause is the immune system’s disorder. Factors such as UV light, medications, hormonal changes, stress, and infections can favor disease or cause relapses.
Examinations: interview, skin and blood tests. Suppose SLE is suspected, additional examination of internal organs.
Treatment: Consistent UV protection, medications (cortisone, immunosuppressants, etc.), stress avoidance, infection prevention
What is lupus erythematosus?
Lupus erythematosus (butterfly lichen) is mainly a relapsing autoimmune disease from collagenosis. These are connective tissue diseases that are considered inflammatory rheumatic diseases.
In the case of an autoimmune disease, the immune system’s defense cells (antibodies) attack the body’s own cellular components and thus cause inflammatory changes. Depending on what these structures are and how the disease progresses, doctors distinguish different erythematous lupus forms. The two most common are:
- Cutaneous lupus erythematosus (CLE)
- Systemic lupus erythematosus (SLE)
There are other, less common forms of lupus. These include, for example, neonatal lupus erythematosus (NLE) and drug-induced lupus erythematosus (DILE).
Cutaneous lupus erythematosus
Cutaneous lupus erythematosus (CLE) occurs mainly at 3. and the 4th decade of life, and more often in women than in men. Data on the incidence of the disease in the population are limited. A Swedish study found four new cases of CLE per 100,000 population.
Most CLE only affects the skin. It occurs in different sub-forms:
- Acute cutaneous lupus erythematosus (ACLE)
- Subacute cutaneous lupus erythematosus (SCLE)
- Chronic cutaneous lupus erythematosus (CCLE) – with three subforms, and the most common is discoid erythematous lupus (DLE).
- Intermittent cutaneous lupus erythematosus (ICLE) – with a subform
The most important variants of CLE include subacute cutaneous lupus erythematosus ( SCLE ) and discoid erythematous lupus (DLE).
Systemic lupus erythematosus (SLE)
In this variant of lupus and the skin, a wide range of internal organs are also affected. For example, inflammation of the kidneys, lungs, and heart is common. Most patients also develop joint pain. Muscles can also be affected. Overall, the disease course can vary greatly from patient to patient.
Systemic lupus erythematosus is most common in young women. The disease can also break out in childhood.
Lupus erythematosus: frequency
Lupus erythematosus is common, but rare, worldwide. Overall, autoimmune disease occurs in about 50 out of 100,000 people. Women of reproductive age are most commonly affected.
Lupus disease symptoms
Which symptoms will occur in lupus erythematosus, crucially depends on the course of the disease. The disease can be limited to the skin, but can also affect the internal organs.
Discoid lupus erythematosus (DLE)
Typically, the appearance of a sharply demarcated, slightly convex disc-shaped redness of the skin, consisting of coarse scales, reveals this form of lupus. Lupus vilest occurs mainly in this form on parts of the body that are often exposed to the sun, such as the nose, forehead, cheeks, lips, earlobes, and the back of the hands. The redness of the skin on the face often looks like a butterfly. That is why lupus erythematosus is also called butterfly disease.
Reddish-scaly changes on the skin spread outwards.
Common symptoms of lupus in the disease’s discoid form are also pit-shaped scars with visibly enlarged small skin vessels (telangiectasia), as well as spotted areas of skin with reduced or increased discoloration (hypo- or hyperpigmentation).
Subacute cutaneous lupus erythematosus (SCLE)
It occupies a middle place between cutaneous lupus (with discoid shape as the most common subgroup) and systemic lupus:
On the one hand, as with the disease’s discoid form, inflammatory changes occur on the skin (bright red, scaly, oval, or ring-shaped), especially on the face, chest, and arms. However, these phenomena are less characteristic than discoid lupus and sometimes resemble psoriasis. Pigmentation disorders are sporadic.
On the other hand, internal organs can also be affected by subacute cutaneous lupus erythematosus, and specific antibodies can be detected in the blood. These two lupus symptoms are otherwise typical of systemic lupus erythematosus.
Systemic lupus erythematosus
The diverse clinical picture of systemic lupus erythematosus includes, for example, skin rashes (often in the form of butterflies on the face), painful and/or inflamed joints and muscles, as well as inflamed tendon sheaths (tendovaginitis). Also, signs of inflammation of the internal organs often develop (e.g., pleurisy, myocarditis, pericarditis, inflammation of the kidneys).
Lupus erythematosus: how dangerous is the disease?
According to current knowledge, there is no cure for cutaneous lupus erythematosus. With proper therapy, including UV protection of the skin, you can drastically reduce the symptoms.
Different forms of cutaneous lupus erythematosus can develop into systemic lupus with different frequencies. For example, in discoid lupus erythematosus (DLE), it occurs in less than five percent of cases, in subacute cutaneous lupus erythematosus (SCLE), however, in ten to 15 percent of cases.
The course and prognosis of systemic lupus erythematosus (SLE) primarily depends on which internal organs are affected and to what extent. If the kidneys, heart, and lungs are involved, SLE often takes an uncertain treatment course. In individual cases, lupus can even be fatal. However, in Germany, most SLE patients have a normal life expectancy.
Lupus erythematosus: causes and risk factors
The exact causes of lupus erythematosus are not yet fully known. According to experts, genetic predisposition is the basis for the immune system disorder on which the disease is based. In combination with other factors, lupus can occur. The factors are as follows:
First of all, UV radiation should be mentioned here. Another possible influencing factors are hormonal influences because erythematous lupus occurs much more often in women and girls than in men and boys (hormonal balance in women is subject to greater fluctuations than in men). Also, other factors such as stress and infections can cause the disease to worsen and progress.
Lupus erythematosus: examinations and diagnosis
At the beginning of the diagnosis of lupus erythematosus, the doctor will talk in detail with the patient (in the case of children with parents) about the anamnesis. For example, he will ask what symptoms are present when they first appeared and whether the patient has any other disease in addition to those symptoms. A physical examination follows this, usually followed by further analysis.
Skin examination
Typical changes in the skin occur in various forms of lupus. Therefore, a lupus test by a dermatologist is important for diagnosis. The doctor takes a tissue sample (skin biopsy) from the affected areas of the skin to do this. The sample is examined in more detail in the laboratory using different methods.
Further investigations
In cutaneous lupus erythematosus (CLE), standardized photo provocations may be useful in special cases. The skin is especially exposed to UV light to check if it reacts to typical CLE damage. They appear on average eight days (plus / minus 4.6 days) after exposure to UV radiation and then last for a longer period of time. Photo provocation can help, for example, in distinguishing CLE from polymorphic light dermatosis (skin damage caused by UV radiation occurs earlier and then recedes).
Blood tests can also provide important indications of autoimmune disease. For example, in systemic lupus erythematosus and most cases of subacute cutaneous lupus erythematosus, specific antibodies can be detected in the blood.
Also, whenever lupus is suspected, it must be clarified whether the disease also affects the internal organs. If this is the case, this suggests systemic lupus erythematosus. Useful examinations may be, for example, imaging procedures (such as X-rays or ultrasounds), fundus examinations, or lung function tests.
Lupus erythematosus: treatment
Treatment of lupus erythematosus depends on the form and severity of the disease.
Cutaneous lupus erythematosus: therapy
Skin changes affected by lupus are treated externally (local therapy). In some cases, patients also need to take medication (systemic therapy). Also, other measures can positively affect the course of the disease.
Local therapy
Using local therapy, inflammatory changes in cutaneous lupus erythematosus are treated externally:
Topical glucocorticoids (“cortisone”): Localized areas with skin changes are preferably treated with topical cortisone preparations (e.g., cortisone ointment). The application should be as short as possible due to possible side effects.
Local calcineurin inhibitors: These are immunosuppressants, i.e., substances that have a calming effect on the immune system (e.g., Tacrolimus). They are recommended primarily for the local treatment of skin changes on the face and as an alternative to local cortisone preparations.
Local retinoids: Topical treatment with these vitamin A acid derivatives (such as tazarotene, tretinoin) is an option in severe cases of cutaneous lupus erythematosus.
Cold treatment, laser therapy: If other treatment measures against skin changes do not help, you may consider cold treatment (cryotherapy) or laser therapy in special cases.
Systemic therapy
It may be necessary to take additional medications if patients do not respond to the basic medications or if the skin damage is very pronounced. The following groups of active substances are available:
Antimalarial drugs: Active ingredients such as chloroquine or hydroxychloroquine are among the most important basic skin lupus remedies. Due to the risk of retinal damage, the patient should have regular eye examinations by an ophthalmologist during treatment.
Glucocorticoids: you should limit cortisone use in time due to possible side effects. It would help if you stopped it as soon as possible by gradually reducing the dose (narrowing the therapy).
Other immunosuppressants: In addition to cortisone, other immunosuppressants may be given for skin lupus. For example, methotrexate (MTKS) is considered the drug of choice in persistent cases of subacute skin lupus (SCLE) and discoid lupus (DLE). If possible, it is used together with antimalarial drugs. Other immunosuppressants for skin lupus include azathioprine and cyclosporine.
Retinoids: In certain skin lupus cases, taking retinoids may be helpful. It is desirable to use them with antimalarial drugs.
Dapsone: This bacterial and anti-inflammatory agent may be prescribed, for example, to treat a bullous form of cutaneous lupus erythematosus.
In certain cases, the doctor may prescribe other drugs to be taken, for example, strong anti-inflammatory drugs thalidomide or belimumab – a therapeutic antibody against certain immune cells.
You should not use certain drugs (e.g., retinoids) in pregnant or breastfeeding patients. The doctor must take this into account when planning the therapy.
Also, vitamin D. is prescribed for patients with lupus when they are deficient. This is actually one of the risk factors for developing cutaneous lupus erythematosus and systemic lupus erythematosus. If the deficiency is compensated, it can, in some cases, have a positive effect on the course of the disease.
Other measures to treat lupus
Treatment of cutaneous lupus erythematosus includes consistent sun protection: Patients should avoid direct sunlight and use sunscreens with a high protection factor against UV-A and UV-B radiation. Artificial UV sources (e.g., in solariums) are not recommended and should be avoided.
It is also recommended to refrain from active and passive smoking. Nicotine consumption is a risk factor for erythematous lupus of the skin.
In some cases of subacute cutaneous lupus erythematosus (SCLE), it may help avoid certain medications (in consultation with the treating physician!). Some drugs can promote this form of lupus, such as the anti-fungal drug terbinafine, the diuretic hydrochlorothiazide, and various calcium channel blockers used to treat high blood pressure (such as verapamil).
