Lupus erythematosus

from Wikipedia, the free encyclopedia
Classification according to ICD-10
M32.- Systemic lupus erythematosus
M32.0 Drug-induced systemic lupus erythematosus
M32.1 + Systemic lupus erythematosus with involvement of organs or organ systems
M32.8 Other forms of systemic lupus erythematosus
M32.9 Systemic lupus erythematosus, unspecified
L93.- Lupus erythematosus
L93.0 Discoid lupus erythematosus

Lupus erythematosus onA

L93.1 Subacute cutaneous lupus erythematosus
L93.2 Other localized lupus erythematosus

Lupus erythematosus profundus
lupus panniculitis

ICD-10 online (WHO version 2019)
Butterfly erythema in systemic lupus

The lupus erythematosus ( Latin lupus , wolf ' , and ancient Greek ἐρυθηματώδης erythematosus from ἐρύθημα erythema , German , redness' , and suffix -ώδης -ṓdēs , German , similar to' ), including lupus and epidermolysis bullosa is a rare autoimmune disease that in different Forms and subforms occurs:

The systemic lupus erythematosus (SLE) is a rheumatic disease from the group of collagen and may all organs affected.

Neonatal lupus erythematosus can occur in newborns of mothers with lupus .

The cutaneous lupus erythematosus (CLE) is a dermatological disease, it usually affects only the skin .

Lupus erythematosus occurs in families. It is predominantly women of childbearing age that become ill. Lupus erythematosus (untreated) usually takes place in phases, i.e. in regularly recurring active phases. There can be long phases in between the attacks in which the disease is not or only little active. Butterfly erythema is particularly characteristic of lupus erythematosus , a reddening that extends symmetrically from the bridge of the nose to the cheekbones and cheek regions.

origin of the name

Lupus erythematosus, colored photo from 1886

The name Lupus was introduced by the Lombard surgeon Roger Frugardi (around 1140–1195), but is also documented in the 10th century. The term lupus is derived from the Latin name for the wolf. In the past, the scars that remained after the skin damage had healed were compared with scars from wolf bites. Today, thanks to modern treatment options, such disfiguring scars rarely occur. The word erythematosus means "blushing" and is derived from the reddening of the skin of the butterfly erythema , which is common in this disease.


The disease has been known at least since the Middle Ages . The French Pierre Louis Alphée Cazenave (1795–1877) was the first to describe the disease in a scientifically accurate form in 1851.

In 1872 the disease was described by the Hungarian dermatologist Moritz Kaposi .

Cause and origin

Frequency of positive ANA titers in healthy subjects
01:40 32% (- 40%)
01:80 13% (- 32%)
01: 160 05% (- 16%)
01: 320 03%

A genetic predisposition as well as endogenous and exogenous triggers play a role in the disease .

The normal immune reaction in the autoimmune disease lupus erythematosus is dysregulated: It is permanently activated and continuously produces an increased number of antibodies against the body's own cell nucleus components, so-called antinuclear antibodies (ANA). These autoantibodies damage the body's own cells.

As part of the cell damage caused by the autoantibodies , cells die. The dead cells disintegrate so that their core components are released. As a result, the cell nucleus components of healthy endogenous cells are recognized as foreign by the malfunctioning immune system. In healthy people, dead cells are eliminated immediately so that cell nucleus components are not visible to the immune system. The immune system reacts to the cell nucleus components of the body's own cells, which are wrongly recognized as foreign, and the body's own immune system is activated and further antinuclear antibodies are produced. The activation of the immune system triggers an inflammatory reaction and thus additionally damages the respective tissue .

Immune complexes are formed from cell nucleus components and autoantibodies. These can not be eliminated by the reticulohistiocytic system , part of the body's immune system, and are deposited in a wide variety of places, e.g. B. on the walls of the blood vessels. This increased arteriosclerosis can lead to a vascular narrowing or a vascular occlusion, so that the organs or organ systems are only insufficiently supplied with blood and can be additionally damaged. The deposits of immune complexes on the walls of the blood vessels also lead to inflammatory reactions. These can cause vasculitis .

The exact cause of this pathological reaction of the immune system is still unknown. However, it is assumed, among other things, that an increased interferon activity of type I interferon plays an important role. About 80% of SLE patients have what is known as an IFN signature when examining blood cells. The detection of IFN in the blood is very difficult due to the many subtypes, which is why the indirect detection method is chosen. Various proteins are available here that are produced in response to interferon. One that has now been well researched is IP-10, another is SIGLEC1. The interferon signature, read out via such proteins, is mainly determined in studies or z. Sometimes also in routine diagnostics. The level of the IFN signature correlates with the level of disease activity.

Cutaneous Lupus Erythematosus (CLE)

In cutaneous lupus erythematosus ( Latin cutis = skin) (CLE), also lupus erythematosus cutaneus or skin lupus, usually only the skin is affected. Systemic lupus erythematosus (SLE) can develop from all forms of cutaneous lupus erythematosus (CLE) .

Different systems are used to systematize the various forms of cutaneous lupus erythematosus (CLE). This results in a different use of the disease names and abbreviations. In the literature, the term CLE is also used for the skin manifestation of systemic lupus erythematosus (SLE). The AWMF guidelines define cutaneous lupus erythematosus (CLE) as a separate clinical picture in order to standardize the terminology and system. The systematization here follows the systematization of the AWMF guidelines, the so-called Düsseldorf classification of the dermatologists James N. Gilliam and Richard D. Sontheimer.

In addition to the classic forms of cutaneous lupus erythematosus (CLE) listed here, there are other rare variants that are no longer listed as separate subtypes, but are assigned to the classic forms.


The frequency of the disease ( prevalence ) in Europe is less than 50 per 100,000 inhabitants. This corresponds to around 40,000 people with cutaneous lupus erythematosus (CLE) in Germany. Women get it three to six times more often than men. In lupus erythematosus tumides (LET), the gender ratio is balanced.

to form

Acute cutaneous lupus erythematosus (ACLE)

The term Erythematodus acutus is an outdated term for systemic lupus erythematosus.

The skin symptoms of systemic lupus erythematosus (SLE), butterfly erythema , skin rash, changes in the mucous membrane can also occur without further organ involvement and are referred to by some authors as acute cutaneous lupus erythematosus (ACLE). If other organ involvement occurs in addition to the skin symptoms, the disease is classified as systemic lupus erythematosus (SLE) according to the ICD-10 classification of the WHO and the AWMF guidelines .

Subacute Cutaneous Lupus Erythematosus (SCLE)

Subacute cutaneous lupus erythematosus (SCLE), also known as subacute cutaneous lupus erythematosus, is a rare form of lupus erythematosus. It has an intermediate position between cutaneous lupus erythematosus (CLE) and systemic lupus erythematosus (SLE). In subacute cutaneous lupus erythematosus (SCLE), the ACR criteria for the diagnosis of systemic lupus erythematosus (SLE) are formally met in about 50% of cases.

Subacute cutaneous lupus erythematosus (SCLE) shows psoriasis- like round foci that can flow into one another, or similar papulosquamous skin changes. Rarely does a TEN- like picture appear, i.e. a blistered detachment of the epidermis. The skin changes usually appear symmetrically in areas exposed to light or in a generalized manner . In contrast to chronic discoid lupus erythematosus (CDLE), these skin conditions usually do not scar, but can leave behind pigmentation disorders . A butterfly erythema is possible. In addition, joint and muscle pain, rarely also kidney involvement and involvement of the central nervous system, with mild symptoms, have been described.

Subacute cutaneous lupus erythematosus is often triggered by drugs. Then it usually heals after stopping the triggering drug. Subacute cutaneous lupus erythematosus, as a so-called paraneoplastic dermatosis, can also be associated with carcinomas or Hodgkin's lymphoma .

Chronic cutaneous lupus erythematosus (CCLE)

Chronic Discoid Lupus Erythematosus (CDLE)
The singer Seal : The scars of his lupus erythematosus are clearly visible.

Chronic discoid lupus erythematosus (discus in Greek δίσκος = disk) (CDLE), also called discoid lupus erythematosus (DLE), is by far the most common form of cutaneous lupus erythematosus (CLE) and is therefore often used as a generic term for the other forms of skin lupus and often referred to as skin lupus itself.

Chronic discoid lupus erythematosus (CDLE) is mainly localized on light-exposed skin areas, v. a. Face, scalp, décolleté, extensor sides of the extremities , upper body. Changes in the area of ​​the red lips and cheek mucosa also occur. It can be triggered by irritative stimuli ( Koebner phenomenon ).

The skin changes of chronic discoid lupus erythematosus (CDLE) usually manifest themselves in the form of discs. These skin changes are often sensitive to touch and have a three-phase structure:

  1. There is a reddening at the edge, which in one
  2. scaly area passes over. The skin flakes are stuck. After removing a scale, a so-called keratotic spur is found on the underside of it (wallpapering nail phenomenon ).
  3. There is a central tissue loss ( atrophy ).

A butterfly erythema is possible. The skin changes usually heal with scarring and / or hypopigmentation . This can lead to mutilation of the nose and mouth. Squamous cell carcinoma rarely develops in the scars . In hairy areas of the skin, the scars can lead to permanent circular hair loss ( alopecia ).

Lupus erythematosus profundus (LEP)

Lupus erythematosus profundus ( Latin: profundus = deep) (LEP), also called lupus panniculitis, is a rare form of cutaneous lupus erythematosus (CLE) with solid lumps in the subcutaneous fatty tissue of the face, shoulders, buttocks and extremities. The overlying skin may look normal, be reddish or have skin changes of chronic discoid lupus erythematosus (CDLE). Deep atrophic scars may remain as it heals.

Photosensitivity does not usually occur in lupus erythematosus profundus (LEP).

Chilblain Lupus Erythematosus (CHLE)

In the rare chilblain lupus erythematosus (CHLE), also called chilblain lupus ( English chilblain = frostbite), symmetrical bluish-red swellings that are painful on pressure occur, which can turn into large, limited, cushion-like knots. The demarcation of frostbite is difficult. Chilblain lupus erythematosus (CHLE) mostly occurs in the damp and cold season. Mostly cold-exposed areas are affected, i.e. toes, heels, soles of the feet, fingers, ears and nose. Photosensitivity does not usually occur in Chilblain Lupus Erythematosus (LEP).

Intermittent Lupus Erythematosus (ICLE)

Lupus erythematosus tumidus (LET)

The skin lesions of the rare lupus erythematosus tumidus (LET) are mostly located in sun-exposed areas. Clinically, sharply demarcated, reddish papules with mostly glossy surfaces, which are often arranged in a circle or crescent shape, appear. These usually heal without scarring or pigmentation changes. Spontaneous regressions are also possible.


Lupus band
Autoantibodies of the subtypes of CLE
Autoantibodies ACLE SCLE CDLE LET
ANA +++ ++ + (+)
Anti-ds-DNS +++ 0 0 0
Anti-sm ++ 0 0 0
Anti-Ro / SSA + - ++ +++ 0 (+)
Anti-La / SSB (+) ++ (+) 0 (+)
+ = Frequency of presence

The anamnesis , the clinical and dermatological examination often already enable the assignment to a certain form of cutaneous lupus erythematosus (CLE). The skin changes of cutaneous lupus erythematosus (CLE) may also have to be provoked by a photo provocation for diagnosis .

The diagnosis is supplemented and confirmed by a tissue examination and the determination of the immunofluorescence ( DIF ) of a skin sample: Examination under the microscope reveals specific skin changes typical for the respective subtype of cutaneous lupus erythematosus (CLE) in the examined skin areas.

When determining the immunofluorescence, dye-labeled antibodies are used that bind to the body's own antigens . This enables the accumulation of these antigens along the basement membrane of the skin to be seen under the immunofluorescence microscope in lupus erythematosus . Since the basement membrane separates the epithelium from the connective tissue , the image of a luminous band, the so-called lupus band, is created.

Laboratory tests of the blood and urine are necessary to further supplement and confirm the diagnosis and to exclude or prove organ involvement .

Depending on the course and symptoms, additional diagnostic equipment may be necessary to check whether organ involvement is present.


The diagnosis of lupus erythematosus in itself is not a reason for drug treatment.

When individual skin areas are affected, glucocorticoids or retinoids applied externally as an ointment can be effective. Calcineurin inhibitors are particularly suitable for treatment on the face and for longer treatments . Resistance to therapy, extensive skin involvement, the risk of scarring or pigmentation disorders often make internal ( systemic ) therapy necessary for cutaneous lupus erythematosus (CLE) .

Systemic lupus erythematosus (SLE)

Common symptoms of systemic lupus erythematosus

The systemic lupus erythematosus (SLE), also lupus erythematosus disseminatus (from Latin disseminare = to sow) (LED) or obsolete lupus erythematosus visceralis (visceral lupus erythematosus) as well as Kaposi-Libman-Sacks syndrome and popularly called the generalized form of butterfly disease is called the generalized disease Lupus erythematosus. The SLE can attack and seriously damage any organ and organ system. Since the late stage of the disease can almost always be avoided today with medication, the full picture only occurs very rarely.


The frequency of the disease ( prevalence ) is around 25–27 per 100,000 inhabitants in Europe, and around 37 per 100,000 in Germany. For Germany this corresponds to around 30,000 people with systemic lupus erythematosus. The frequency of new cases ( incidence ) is given as 6–8 per 100,000 inhabitants and year.

The number of new cases has tripled in the last four decades. One reason for this is likely to be in improved diagnostics. New cases are particularly common in spring and summer.

With systemic lupus erythematosus around 90% of all sufferers are women. In 2002, however, the proportion of women diagnosed with SLE in Germany was only around 80%.


Overview of possible symptoms in systemic lupus erythematosus
Frequency in% symptom
085 Joint pain
084 General complaints
(tiredness, poor performance ...)
081 Skin changes
077 Kidney findings
063 Joint inflammation
058 Raynaud's Syndrome
054 Complaints d.
Central nervous system
054 Mucosal changes
047 Gastrointestinal complaints
037 pleurisy
032 Lymph node disease
029 Pericarditis
017th Lung involvement
005 Muscle inflammation
004th Myocarditis
004th Inflammation of the pancreas

Depending on which organs or organ systems are affected, the symptoms of systemic lupus erythematosus (SLE) vary widely and can change over the course of the disease. For systemic lupus erythematosus there are therefore no sure and obligatory key symptoms. The symptoms described here do not all occur in all sufferers, but in different combinations. They can occur simultaneously or at different times. In addition, systemic lupus erythematosus (SLE) has a large number of other possible symptoms and a large number of possible secondary diseases.

  • General symptoms: General symptoms such as tiredness, exhaustion and / or poor performance as well as subfebrile temperatures are very common accompanying symptoms of systemic lupus erythematosus (SLE).
  • Skin: The skin symptoms of systemic lupus erythematosus (SLE) can be very diverse: About 80% of butterfly erythema occurs in systemic lupus erythematosus (SLE). A maculopapular rash is very common, so a measles-like rash that can affect the entire body. These skin symptoms usually heal without scars. All skin symptoms of cutaneous lupus erythematosus (CLE) can occur together with systemic lupus erythematosus.
  • Mucous membranes: there are often mostly painless open wounds on the mucous membranes of the mouth and / or nose. Inflammation of the lips ( cheilitis ) can also occur.
  • Hair: Diffuse hair loss ( alopecia ) is another common symptom of systemic lupus erythematosus.
  • Joints and muscles: In addition, many sufferers complain of rheumatoid-like joint and / or muscle pain. In most cases, the joints are not destroyed, as is the case with other rheumatic diseases. The connective tissue of the joints, tendons and muscles can also become weak. This can lead to deformations or malpositions, for example to a Z-shaped deformation of the thumb ( lupus arthropathy ).
  • Kidneys: Approx. 70% of people with systemic lupus erythematosus (SLE) develop lupus nephritis through involvement of the kidneys . This can be symptom-free and can then only be detected by urine examination and / or kidney biopsy. Lupus nephritis determines the prognosis for the course of systemic lupus erythematosus .
  • Nervous system: manifestations of the central nervous system occur in around 15–50% of patients with SLE and are difficult to differentiate because of their non-specificity (e.g. headache) and high variability. Localized damage in the central nervous system (more likely neurological symptoms, often due to circulatory disorders in the case of antiphospholipid syndrome), as well as diffuse involvement of the central nervous system (more psychiatric symptoms) and involvement of the peripheral nervous system are possible . It can migraine-like headaches, cognitive impairment, epileptic seizures, stroke , incoordination , psychosis , depression occur or confusion.
  • Lungs / pleura : In 75% of patients, inflammation of the pleura or lungs ( pleurisy ) occurs in the course of the disease . The accumulations of fluid ( exudates ) can (with polyserositis in up to 50% of cases) cause a pleural effusion and cause severe pain when breathing. As the disease progresses, pulmonary fibrosis can develop, which can cause shortness of breath during exertion.
  • Heart: A pericarditis , an inflammation of the endocardium or heart muscle inflammation may occur with relevant cardiac symptoms.
  • Digestive system / peritoneum: The digestive system can be attacked by systemic lupus erythematosus. However, peritonitis can also lead to stomach pain, nausea and vomiting.
  • Blood vessels: Vasculitis , an inflammation of the small blood vessels, can show up as bleeding into the skin or mucous membrane (red spots or spots).
  • Eyes: Inflammation of the blood vessels in the fundus can lead to blurred vision and visual field defects. Changes in the smallest of vessels ( microangiopathy ) can also lead to thrombosis of the retinal veins with a subsequent, often irreversible, reduction in vision. In a Taiwanese cohort study, this was observed 3.5 times more often than in healthy individuals. Younger sick people and those with high blood pressure are particularly at risk, in two thirds of all cases in the first four years after diagnosis. Regular ophthalmological checks are therefore recommended.
  • Blood: Autoantibodies can destroy red blood cells ( erythrocytes ); for the same reason, leukocytes , lymphocytes and platelets in the blood count may also be reduced.

Secondary diseases

  • The circulatory disorders of systemic lupus erythematosus can manifest themselves in the form of Raynaud's syndrome .
  • In some of those suffering from systemic lupus erythematosus there is an overlap with Sjögren's syndrome .
  • About 40% of the sick have an antiphospholipid syndrome in addition to their underlying disease .
  • Sometimes Hashimoto's thyroiditis , an autoimmune disease of the thyroid gland, occurs with systemic lupus erythematosus.
  • Systemic lupus erythematosus (SLE) can be the cause of a type III allergy , an allergy caused by the formation of immune complexes .


In order to differentiate it from other autoimmune diseases, the American College of Rheumatology (ACR) developed diagnostic criteria in 1982 to facilitate the diagnosis of systemic lupus erythematosus , the so-called ACR criteria, formerly ARA criteria. In 1997 these criteria were revised. If four of the eleven criteria are present, at the same time or at different times, the diagnosis of SLE can be made with a certainty of 96%. The eleven ACR criteria are:

  1. Butterfly erythema
  2. Cutaneous lupus erythematosus (Note: Not to be confused with CLE or CDLE, obviously the various skin manifestations of SLE are meant.)
  3. Photosensitivity
  4. Mucosal ulceration in the mouth and / or nose
  5. Joint pain (non- erosive ) ( Note: In the English-language literature, the term arthritis is used for joint pain regardless of its cause.)
  6. Nephritis (cylinder or proteinuria > 500 mg / day)
  7. Encephalopathy ( cerebral seizures or psychosis )
  8. Pleurisy or pericarditis
  9. Anemia , leukopenia, or thrombopenia
  10. Detection of anti-nuclear antibodies (ANA)
  11. Detection of antibodies against double-stranded DNA (dsDNA-AK) or Smith antigen antibodies (Sm-AK).

Since the ACR criteria include four skin criteria alone, they are only of limited suitability for differentiating between systemic lupus erythematosus (SLE) and skin lupus (CLE). Systemic lupus erythematosus (SLE) can be very difficult to diagnose, so it is not justified to call diagnostic efforts dispensable.

There are also very mild forms of the disease in which only one or two ACR criteria are met. After years of progress, these forms of development can turn into the full picture of systemic lupus erythematosus (SLE).

Many people with autoimmune diseases have more than one target organ. This is why it is also possible for different autoantibodies to overlap. For example, in a patient with systemic lupus erythematosus (SLE) and rheumatoid arthritis, both antinuclear antibodies (ANA) and rheumatoid factors are found in the serum without cross-reactions .


Lupus erythematosus is currently not curable. The SLE should be checked regularly and for life by an experienced internal rheumatologist (specialist in internal medicine and rheumatology). The aim of therapy is to prevent or reduce the worsening of the disease and consequential damage as well as the limitations associated with the disease.

The drug therapy of systemic lupus erythematosus is structured in stages and depends on the severity of the symptoms, the current disease activity, the involvement of organs and the effectiveness in the individual patient. In the case of mild illness, symptomatic treatment with so-called non - steroidal anti-inflammatory drugs (NSAIDs) makes sense; these remedies have mild anti-inflammatory and analgesic effects. Hydroxychloroquine lowers disease activity, organ damage and mortality and should therefore be taken by every SLE patient who can tolerate it.

In acute episodes of illness, glucocorticoids (cortisone) are often used as shock therapy. High doses are given over a short period of time. Glucocorticoids have the advantage over other immunosuppressants that their anti-inflammatory effects develop after a few hours to days and that they can be tolerated by everyone. After the episode, the dose is gradually reduced again, as far as the disease activity allows. The aim is to reduce it to zero or at least below the Cushing threshold (in the case of prednisolone less than 7.5 mg per day). In long-term glucocorticoid therapy, especially if the Cushing threshold is not fallen below, the intake of vitamin D is indicated to prevent the development of osteoporosis .

In moderate cases, immunosuppressants such as azathioprine , methotrexate (MTX) or ciclosporin are used. These drugs have to be crept in and often take weeks or months to fully develop their effect.

If the disease threatens to destroy a vital organ (such as the kidney), bolus therapy with the cytostatic drug cyclophosphamide or, alternatively, mycophenolate mofetil (MMF) is often helpful. In severe cases, thalidomide (Contergan) is also used under strict supervision .

Combinations of immunosuppressants, especially with hydroxychloroquine and a glucocorticoid, are possible. The monoclonal antibody belimumab has been approved as a supplement to standard therapy since 2011 ; the effect occurs after three months at the earliest. Common to all immunosuppressants is an increased susceptibility to severe infections. Also, opportunistic infections occur or latent infections flare up again. However, the previous assumption that the immunosuppressive drugs used to treat systemic lupus erythematosus (SLE) increase the risk of malignant tumors, especially of the lymphoid tissue (lymphomas), was confirmed in an international study with over 5000 lupus patients refuted: The risk of developing malignant lymphoma for lupus patients is neither increased by increased disease activity nor by immunosuppressants.

Since SLE massively increases the risk of atherosclerosis , the risk factors of smoking , high blood pressure and lipid metabolism disorders should be consistently contained. This not only prevents heart attacks and strokes ; Monitoring blood pressure in particular also helps to maintain kidney function.

Other possible treatments are immunoadsorption , plasmapheresis , stem cell transplantation , immunoglobulins, and physical therapies .

Course and prognosis

Systemic lupus erythematosus (SLE) usually begins slowly. Most often it runs in spurts in regularly recurring active phases. There can be long phases in between the attacks in which the disease is not or only little active. The damage that occurred during the episode can partially regress during the so-called remission phases. Since the damage is not completely regressed, the disease is progressive overall.

But there are also creeping, permanently active, slowly progressing forms. This form is particularly difficult to diagnose.

Systemic lupus erythematosus rarely begins suddenly and acutely and progresses rapidly , i.e. difficult and rapidly. In the past, this form of disease often and quickly led to death. Thanks to modern treatment methods, it has become very rare today.

The 5-year survival rate today is around 95% with appropriate treatment. Most sufferers do not die from systemic lupus erythematosus (SLE) itself, but from complications, e.g. B. Thromboses and infections . If the disease is recognized early and treated appropriately, most people have a life expectancy that is close to normal.

Drug-induced systemic lupus erythematosus

The drug-induced systemic lupus erythematosus is also known as pseudo-lupus erythematosus syndrome.

Some drugs, e.g. B. hydrazine , hydantoine , procainamide , sulfasalazine , a disease pattern very similar to systemic lupus erythematosus can be triggered. Laboratory parameters for this are antinuclear antibodies (ANA) and histone antibodies. This clinical picture usually subsides quickly after the drug is discontinued.


Lupus erythematosus is often triggered, entertained, and / or exacerbated. These triggers are individually different and can be, for. B. physical, mental, emotional or social stress , infections , hormones and hormone changes, light exposure or drugs. It has been known since 2018 that certain types of bacteria also act as triggers. This concerns Enterococcus gallinarum , a pathogen from poultry fattening, as well as Lactobacillus reuteri , which is used as a livestock probiotic in factory farming and has subsequently also found its way into human medicine as a probiotic against oral diseases, diarrhea and gastric mucosal inflammation.

If the disease progresses relapses, these triggers can trigger further relapses. Those affected often experience disadvantages and a certain stigmatization in their social environment.

Light exposure and photosensitivity

Light exposure is a frequent trigger of the disease lupus erythematosus itself and of relapses when the disease progresses.

A great number of people with lupus erythematosus have a pronounced photosensitivity : The skin symptoms of lupus erythematosus can be provoked, intensified or entertained by light. In addition, the photosensitivity of lupus erythematosus can manifest itself in skin changes similar to sunburn even after low exposure to light and in a burning sensation on the skin without visible reddening. Skin reactions can occur with a delay of up to three weeks after exposure to light.

Some of the drugs required for lupus erythematosus have a photosensitizing effect and can trigger, entertain or intensify the photosensitivity of lupus erythematosus.

Photosensitivity can also manifest itself in a deterioration in the general condition as well as in weakness, fatigue or joint or muscle pain after exposure to light.

Tertiary prevention

There is no prevention against the disease itself. Prevention aims to avoid an aggravation of the disease and secondary diseases. In the event of relapses, further relapses should be prevented or delayed. It is important to know the individual triggers of the disease and to avoid them.

  • Sun protection: Since UV light can trigger a flare-up in many of those affected and entertain and / or worsen the symptoms of lupus erythematosus, consistent sun protection is necessary for them. Photosensitizing drugs should be avoided after consulting the attending physician.
  • Vitamin D prophylaxis: Vitamin D is formed when the skin is exposed to sunlight. Deficiency leads to osteopenia and osteoporosis in adults . Low vitamin D levels are a risk factor for autoimmune diseases. The lower the vitamin D level, the higher the disease activity. In addition, the vitamin D requirement is increased when taking glucocorticoids (cortisone), which many sick people have to take.
As many sick people have to avoid sunlight, vitamin D prophylaxis is necessary. The consumption of up to 4000 I.E. vitamin D3 daily in adults is considered safe.
  • Avoidance of stress: Physical, mental, emotional or social stress as a frequent trigger should be avoided. Relaxation techniques or psychological support can be helpful.
  • Avoiding tobacco smoking : Smoking is a possible trigger for a flare-up of lupus erythematosus. Active and passive smoking additionally intensifies the arteriosclerosis , which is already aggravated in lupus erythematosus . The effectiveness of some of the drugs required for lupus erythematosus is reduced by active or passive smoking. Lupus erythematosus is usually worse in smokers than in non-smokers. Active and passive smoking should therefore be avoided.
  • Medicines: Medicines that trigger relapses should be avoided after consulting the attending physician. Probiotics are recommended on the one hand to protect the intestinal mucosa against the penetration of relapse-triggering antigens, but when using and selecting them, one should consider that probiotic bacterial preparations from Lactobacillus reuteri are now among the possible triggers of lupus.
  • Protection against infection and vaccinations: Consistent protection against infection is necessary for people who are ill who take immunosuppressive drugs. By reducing the body's defenses, even mundane infections can become life-threatening. Protection against infection also includes avoiding crowds during infection times.
Comprehensive vaccination protection is particularly important for people suffering from lupus erythematosus who take immunosuppressive drugs. Vaccination with live vaccines is mostly contraindicated .

Contraception and hormone replacement therapy for lupus erythematosus

Pure estrogen supplements increase the risk of disease and exacerbation of systemic lupus erythematosus (SLE) or a chronic discoid lupus erythematosus (CDLE). Combination preparations with progestins seem to have a more protective effect. For those suffering from lupus erythematosus, therefore, in addition to non-hormonal contraception, the lowest possible dose of gestagens or possibly low-estrogen combination preparations can be considered.

In 2005 several studies were able to show that under certain conditions it can be safe for a small group of sick people to take estrogen-containing preparations.


Pregnancy in SLE patients is always a high risk pregnancy . Many drugs for the treatment of SLE (immunosuppressants such as mycophenolate mofetil and cyclophosphamide , but also ACE inhibitors and sartans for controlling blood pressure) must not be taken during pregnancy; higher doses of glucocorticoids should be avoided. In order to prevent relapses during pregnancy as much as possible, SLE should be stabilized before the start of pregnancy with medication that can be continued during pregnancy. These are in particular hydroxychloroquine , if necessary also azathioprine and cyclosporine . The pregnancy must be closely monitored by a gynecologist and a rheumatologist or nephrologist, with blood pressure monitoring being particularly important. Pregnancy with active lupus nephritis is strongly discouraged as the risk of preeclampsia is up to 60%. The following risk factors should also be considered:

Antiphospholipid Syndrome

A antiphospholipid syndrome increases the risk of miscarriage and preeclampsia significantly. It should therefore be treated with ASA and low molecular weight heparin during pregnancy .

Neonatal lupus erythematosus

A neonatal lupus erythematosus may SSB antibodies occur in children of mothers with SSA or, as these via the placenta to be transferred to the child. SSA or SSB antibodies occur in lupus (especially in SCLE), but also in Sjögren's syndrome . A transfer is possible from the 16th week of pregnancy. The mother herself may still be symptom-free at this point.

Symptoms in children are skin changes similar to those of subacute cutaneous lupus erythematosus (SCLE). Also can myocardial fibrosis with AV block occur. This heart disease in the child is acquired during pregnancy ( congenital heart block ). It can not be reversed even with immunosuppressive therapy and usually has to be treated with a pacemaker .

With the exception of the AV block on the heart, the disease has a good prognosis because the other symptoms disappear within two years as the autoantibodies passively transmitted by the mother are broken down.

Only a small proportion of children born to mothers with systemic lupus erythematosus (SLE) or Sjogren's syndrome develop neonatal lupus erythematosus. In a prospective study, skin changes were found in 16%, an AV block III. Degree described in 1.6%, increased liver values ​​in 26% and haematological abnormalities in 27% of the cases. Symptoms in other organs such as the kidneys, lungs, nervous system or blood cells are much less common.


  • Through the television series Dr. House became known to the wider public as a disease as the (rejection of) diagnosis of lupus erythematosus became a recurring item on the show. The inventor of Dr. House, David Shore , received the Loop Award from the Los Angeles Branch of the Lupus Foundation for raising awareness of lupus erythematosus.
  • Systemic lupus erythematosus is the theme of fiction in Adolf Muschg's 1984 novel Das Licht und der Schlüssel. Educational novel of a vampire .

Web links

Wiktionary: Butterfly lichen  - explanations of meanings, word origins, synonyms, translations
Commons : Systemic Lupus Erythematosus (SLE)  - Collection of pictures, videos and audio files

Individual evidence

  1. P. Altmeyer: Altmeyer: Encyclopedia Dermatology, Lupus Erythematodes. ( Memento of December 14, 2013 in the Internet Archive ) Retrieved September 22, 2013.
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