Lupus erythematosusLupus erythematosus (also called butterfly lichen) is an autoimmune disease with scaly skin lesions . Read more about symptoms, causes and treatment.
Lupus erythematosus is an autoimmune disease. It is therefore triggered by an erroneous reaction of the immune system. Doctors distinguish between three main forms of lupus erythematosus:
– In chronic discoid lupus erythematosus (CDLE), the symptoms appear mainly on the skin. Reddish scaly skin changes are typical, especially on the face. Therefore, this form of lupus is considered a skin disease. Also called skin lupus or butterfly lichen (butterfly erythema). – In systemic lupus erythematosus (SLE), the disease is not limited to the skin but spreads to internal organs. – Subacute cutaneous lupus erythematosus (SCLE) is a mixed form of the two forms of lupus, usually milder than SLE.
Lupus erythematosus can be accompanied by a variety of symptoms. In addition to skin changes, these include muscle and joint pain, as well as pronounced fatigue and poor performance. The symptoms usually occur in episodes. Sunlight and stress are known triggers of such relapses.
Lupus erythematosus is not yet curable. In drug treatment, mainly analgesics are used. Anti-inflammatory drugs used.
Frequency
Lupus erythematosus is a very rare disease. The number of new cases per year (annual incidence) in this country is about 6 cases per 100.000 inhabitants. Affected are mainly young women up to the age of 30. year of life. They account for 90 percent of the. The total number of patients (prevalence) in Europe is estimated at up to 40 cases per 100 years of age.000 inhabitants stated.
Symptoms
The most obvious symptoms of lupus erythematosus appear on the skin. Almost all patients experience episodes of scaly, reddish skin changes in the form of butterfly wings. They gave the disease the name butterfly lupus. In former times one thought rather of a wolf bite. This is where the name lupus (Latin name for wolf) comes from.
In addition to these erythema, a variety of other symptoms are possible, which occur in phases and vary greatly from individual to individual. The episodes of fever are frequent. accompanied by a pronounced feeling of illness.
Mild symptoms of lupus erythematosus include:
– recurring headaches and pain in the limbs as well as inflammation of the joints – strong sensitivity to light (photosensitivity of the skin) – pronounced fatigue and physical weakness (corpse finger syndrome) – gastrointestinal complaints with nausea and vomiting .
In a large proportion of patients with systemic lupus erythematosus, there is also disease of the internal organs. Particularly common are:
– In 75 percent of cases, inflammatory diseases of the heart and/or lungs occur, such as myocarditis, pericarditis or pleurisy – In half of all patients, a special form of kidney inflammation (lupus nephritis) develops, which can lead to absolute kidney failure and the need for dialysis. – Systemic lupus erythematosus often affects the central nervous system and the brain. The spectrum of these symptoms ranges from simple sensory disturbances, tremors and cramps to memory and concentration disorders. Sometimes epileptic seizures, depression or psychosis occur. – Blood count changes are also common. Many patients have too few white blood cells (leukopenia). A deficiency of blood platelets (thrombocytopenia). Often there is also a special form of anemia, the so-called autoimmune hemolytic anemia.
Causes
The exact cause of lupus erythematosus is not known. All that is known is that the immune system is overactive in episodes for unknown reasons. In this case, the immune system produces more antinuclear antibodies (ANA), among other things. These antibodies suddenly attack healthy cells as well. This is how they cause the inflammatory rheumatic symptoms.
According to current research, genetic factors probably play an important role in the predisposition to lupus erythematosus. In addition, however, there need to be triggers that trigger a relapse.
Trigger
The best-known trigger of lupus erythematosus is sunlight. Almost all forms and relapses occur mainly after exposure to sunlight. Another extremely important trigger are hormonal changes. Women are 10 times more likely to be affected by lupus than men. It therefore seems likely that female sex hormones and their fluctuating concentrations are important risk factors for the disease flare-ups.
Treatment
Lupus erythematosus is currently incurable. Treatment must therefore be limited to alleviating the symptoms. Depending on the severity of the symptoms very different agents are used.
Up to 75 percent of patients with lupus erythematosus respond to treatment with the anti-malarial drugs chloroquine or hydroxychloroquine. However, these drugs are not suitable for pregnant and breastfeeding women. Generally, this form of drug therapy is also used only when lower-risk treatments fail to achieve sufficient success.
– To relieve inflammation, ointments and tablets containing non-steroidal anti-inflammatory drugs such as diclofenac, piroxicam, indometacin or felbinac are used first of all. Cortisone preparations (e.g. prednisone shock therapy) or ointments containing cortisone are often prescribed. Ointments help against the effects of light. Creams with high sun protection factors. – If the symptoms of systemic lupus erythematosus are severe, drugs that suppress the immune system are used. These include, for example, azathioprine, ciclosporin A, mycophenolate mofetil, or monoclonal antibodies such as belimumab. – In rare cases, drugs from cancer therapy are used. These include cytostatic drugs such as methotrexate or cyclophosphamide. By the way, pregnant and breastfeeding women should not take this medication. – Since 2012, the cytostatic drug cyclophosphamide (trade name Endoxan) has been approved for the treatment of lupus nephritis.
Prevention
To avoid relapses and worsening of the skin condition, those affected should avoid direct sunlight and always apply sunscreen with a very high sun protection factor (50 or more). In addition, a healthy, stress-free lifestyle with a balanced diet without excessive consumption of alcohol and nicotine helps to reduce relapses. Medications that trigger relapses should be changed by the physician. In addition, adequate protection against infection and vaccinations are important.