Brief overview
- What is lupus erythematosus? Rare chronic inflammatory autoimmune disease that affects mainly young women. Two main forms: Cutaneous lupus erythematosus (CLE) and systemic lupus erythematosus (SLE).
- Symptoms: CLE affects only the skin with typical butterfly-shaped skin changes on parts of the body exposed to the sun, SLE additionally affects internal organs (e.g. kidney inflammation, joint pain).
- Causes & risk factors: The presumed cause is a disorder of the immune system. Factors such as UV light, medications, hormonal changes, stress and infections can promote the disease or trigger relapses.
- Examinations: Collection of medical history, skin and blood examinations. If SLE is suspected, additional examination of internal organs.
- Treatment: Consistent UV protection, medication (cortisone, other immunosuppressants, etc.), avoidance of stress, prevention of infections.
Lupus erythematosus (butterfly lichen) is an autoimmune disease from the group of collagenoses that usually progresses in relapses. These are connective tissue diseases that belong to the inflammatory rheumatic diseases.
- Cutaneous lupus erythematosus (CLE)
- Systemic lupus erythematosus (SLE)
In addition, there are some rarer forms of lupus. These include neonatal lupus erythematosus (NLE) and drug-induced lupus erythematosus (DILE).
Cutaneous lupus erythematosus
CLE usually affects the skin exclusively. It occurs in several subtypes:
- Acute cutaneous lupus erythematosus (ACLE)
- Subacute cutaneous lupus erythematosus (SCLE)
- Chronic cutaneous lupus erythematosus (CCLE) – with three subtypes, the most common being discoid lupus erythematosus (DLE).
- Intermittent cutaneous lupus erythematosus (ICLE) – with one subtype.
Systemic lupus erythematosus (SLE)
In this variant of lupus, various internal organs are affected in addition to the skin. For example, inflammation of the kidneys, lungs and heart is common. Very many patients also develop joint pain. In addition, the muscles can be affected. Overall, the course of the disease can vary greatly from patient to patient.
You can learn more about this form of lupus disease in the article Systemic lupus erythematosus.
Lupus erythematosus: Incidence
Lupus erythematosus is common but rare worldwide. Overall, the autoimmune disease occurs in about 100 out of every 100,000 people (equivalent to 0.1 percent of the population). Women of childbearing age are most commonly affected.
Lupus erythematosus: symptoms
Discoid lupus erythematosus (DLE)
The reddish-scaly skin lesions spread outwards, while they heal slowly from the center with detachment of the scales. A horny plug can be seen on the underside of the detached scales. This so-called “tapestry nail phenomenon” is typical of discoid lupus erythematosus. The skin areas under the detached scales are thin, shiny, white and – on the hairy head – hairless.
Subacute cutaneous lupus erythematosus (SCLE).
It occupies an intermediate position between cutaneous lupus (with the discoid form as the most common subgroup) and systemic lupus:
Second, in subacute cutaneous lupus erythematosus, internal organs may also be affected and specific antibodies may be detectable in the blood – these two lupus symptoms are otherwise typical of systemic lupus erythematosus.
Systemic lupus erythematosus
Read more about the wide range of lupus symptoms associated with this form of the disease in the article Systemic lupus erythematosus.
Lupus erythematosus: How dangerous is the disease?
According to current knowledge, cutaneous lupus erythematosus is not curable. However, with the right therapy, including careful UV protection of the skin, the symptoms can usually be managed well.
The course and prognosis of systemic lupus erythematosus (SLE) depend primarily on which internal organs are affected and to what extent. If the kidneys, heart and lungs are also involved, SLE often takes a severe course. In individual cases, lupus can even be fatal. However, most SLE patients have a normal life expectancy.
Lupus erythematosus: Causes & risk factors
UV light is to be mentioned here in the first place. Other possible influencing factors are hormonal influences, since lupus erythematosus occurs much more frequently in women and girls than in men and boys (in the female sex, the hormone balance is subject to greater fluctuations than in the male sex). In addition, other factors such as stress and infections can also trigger relapses.
Lupus erythematosus: Examinations & Diagnosis
Skin examination
Typical skin changes occur in the various forms of lupus disease. A lupus test by a dermatologist is therefore important for diagnosis. For this purpose, the physician takes a tissue sample (skin biopsy) from the affected skin areas. This is then examined more closely in the laboratory using various methods.
Further examinations
Blood tests can also provide important information about the autoimmune disease. For example, in systemic lupus erythematosus and in most cases of subacute cutaneous lupus erythematosus, specific antibodies can be detected in the blood.
Read more about the comprehensive diagnosis of this form of lupus in the article Systemic lupus erythematosus.
Lupus erythematosus: Treatment
Treatment of lupus erythematosus depends on the form and severity of the disease.
Cutaneous lupus erythematosus: Therapy
Local therapy
By means of local (topical) therapy, the inflammatory skin changes in cutaneous lupus erythematosus are specifically treated externally:
- topical glucocorticoids (“cortisone”): Circumscribed 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 the possible side effects.
- Topical retinoids: Local treatment with these derivatives of vitamin A acid (such as tazarotene, tretinoin) is considered in severe cases of cutaneous lupus erythematosus.
- Cold treatment, laser therapy: If other treatment measures do not help against the skin changes, physicians recommend cold treatment (cryotherapy) or laser therapy in selected cases.
Systemic therapy
- Antimalarials: Agents such as chloroquine or hydroxychloroquine are among the most important basic medications for cutaneous lupus. Because of the risk of retinal damage, regular eye examinations are advisable during treatment.
- Glucocorticoids: The use of cortisone preparations should be limited in time because of the possible side effects. It should be stopped as soon as possible by gradually reducing the dose (tapering of therapy).
- Retinoids: In certain cases of skin lupus, the use of retinoids may be useful. They are also preferably combined with antimalarials.
- Dapsone: This anti-bacterial and anti-inflammatory agent is prescribed by physicians for the bullous form of cutaneous lupus erythematosus (except in Switzerland, where no dapsone drugs are currently registered).
In patients who are currently pregnant or breastfeeding, certain drugs must not be used (e.g. retinoids). The attending physician must take this into account when planning the therapy.
Further measures
The treatment of cutaneous lupus erythematosus includes consistent light protection: patients should avoid direct sunlight and use sunscreens with a high protection factor against UV-A and UV-B radiation. Artificial UV sources (such as in tanning salons) are equally unfavorable.
Abstaining from active and passive smoking is also strongly advised. Nicotine consumption is considered a risk factor for cutaneous lupus erythematosus.
Systemic lupus erythematosus: Therapy
The treatment of systemic lupus erythematosus is more extensive because internal organs are affected in addition to the skin. Which organs are affected and how severe the disease is can vary from patient to patient. Accordingly, the treatment is adapted individually.
You can read more about this in the article Systemic lupus erythematosus.
Lupus erythematosus: Prevention
In addition to stress, this includes above all intense UV light (sun, artificial UV sources such as in solariums). You should avoid these even if you have the disease, because lupus erythematosus makes the skin more sensitive to sunlight.
Taking vitamin D can also be useful as a preventive measure in consultation with a doctor.
You can read about the particularities of vaccination in immunocompromised patients in the article Immunosuppression and vaccination.
Lupus erythematosus and nutrition
A balanced diet can alleviate some of the accompanying symptoms of systemic lupus erythematosus. For example, joint pain can be prevented by regularly adding fish to the diet.
