Lupus erythematosus

and Sabine Schrör, medical journalist

Martina Feichter studied biology with an elective subject pharmacy in Innsbruck and also immersed herself in the world of medicinal plants. From there it was not far to other medical topics that still captivate her to this day. She trained as a journalist at the Axel Springer Academy in Hamburg and has been working for since 2007 - first as an editor and since 2012 as a freelance writer.

More about the experts

Sabine Schrör is a freelance writer for the medical team. She studied business administration and public relations in Cologne. As a freelance editor, she has been at home in a wide variety of industries for more than 15 years. Health is one of her favorite subjects.

More about the experts All content is checked by medical journalists.

Lupus erythematosus (butterfly lichen, butterfly erythema) is an autoimmune disease that occurs mainly in women. The immune system wrongly attacks the body's own cell structures. Most of the time, the disease process is more or less limited to the skin, as is the case with cutaneous lupus erythematosus. Internal organs can also be affected (systemic lupus erythematosus). Read more about the causes, symptoms, diagnosis and therapy of lupus disease here.

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. L93M32

Brief overview

What is lupus erythematosus? Rare chronic inflammatory autoimmune disease that mainly affects young women. Two main forms: cutaneous lupus erythematosus (CLE) and systemic lupus erythematosus (SLE).
Symptoms: CLE only affects the skin 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? Cutaneous lupus has a good prognosis (even if it is incurable). Sometimes it turns into systemic lupus. Life expectancy is then usually normal as well. However, fatal courses are possible.
Causes & Risk Factors: Probable cause is an immune system disorder. Factors such as UV light, medication, hormonal changes, stress and infections can favor the disease or trigger relapses.
Examinations: conversation, skin and blood tests. If SLE is suspected, internal organs are also examined.
Treatment: Consistent UV protection, medication (cortisone, immunosuppressants, etc.), avoidance of stress, prevention of infections

What is lupus erythematosus?

Lupus erythematosus (butterfly lichen) is a mostly relapsing autoimmune disease from the group of collagenoses. These are connective tissue diseases that count among the inflammatory rheumatic diseases.

In the case of an autoimmune disease, defense cells of the immune system (antibodies) attack the body's own cell components and thus cause inflammatory changes. Depending on which structures these are and how the disease progresses, doctors differentiate between different forms of lupus erythematosus. The two most common are:

Cutaneous Lupus Erythematosus (CLE)
Systemic lupus erythematosus (SLE)

There are also a few other, rarer 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 in the 3rd and 4th decades 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.

CLE mostly affects the skin only. It occurs in different sub-forms:

Acute cutaneous lupus erythematosus (ACLE)
Subacute Cutaneous Lupus Erythematosus (SCLE)
Chronic cutaneous lupus erythematosus (CCLE) - with three subtypes, the most common of which is discoid lupus erythematosus (DLE).
Intermittent cutaneous lupus erythematosus (ICLE) - with a sub-form

The most important variants of CLE include subacute cutaneous lupus erythematosus (SCLE) and discoid lupus erythematosus (DLE).

Systemic lupus erythematosus (SLE)

In this variant of lupus, a wide variety of internal organs are affected in addition to the skin. For example, inflammation of the kidneys, lungs and heart is common. A great many patients also develop joint pain. The muscles can also be affected. Overall, however, the course of the disease can vary greatly from patient to patient.

Systemic lupus erythematosus is most common in women in young adulthood. The disease can also break out in childhood.

You can find out more about this form of lupus disease in the article Systemic Lupus Erythematosus.

Lupus erythematosus: frequency

Lupus erythematosus is common, but rare, worldwide. Overall, the autoimmune disease occurs in about 50 out of 100,000 people. Women of childbearing age are most commonly affected.

Lupus erythematosus: symptoms

The symptoms of lupus erythematosus depend largely on the course of the disease. The disease can be limited to the skin, but it can also affect internal organs.

Discoid Lupus Erythematosus (DLE)

Typically, the appearance of disc-shaped, sharply demarcated, slightly raised skin redness that consists of rough scales reveals a flare-up in this form of lupus. Parts of the body that are often exposed to the sun, such as the nose, forehead, cheeks, lips, auricles and the back of the hands, are particularly affected. The reddening of the skin on the face often looks like a butterfly. Therefore, lupus erythematosus is also called butterfly disease.

The reddish-scaly skin changes spread outwards, while they slowly heal from the center with the scales detaching. A horn plug can be seen on the underside of the detached scales. This so-called "wallpapering nail phenomenon" is typical of discoid lupus erythematosus. The areas of skin under the detached scales are thin, shiny, white and - on the hairy head - hairless.

Frequent lupus symptoms in the discoid form of the disease are also pit-shaped scars with visibly enlarged small skin vessels (telangiectasias) and blotchy areas of skin with reduced or increased coloration (hypo- or hyperpigmentation).

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:

On the one hand, as with the discoid form of the disease, inflammatory skin changes (light red, scaly, oval or ring-shaped) occur, especially on the face, chest and arms. However, they are less characteristic than discoid lupus and sometimes resemble psoriasis. Scarring and pigmentation disorders rarely occur.

On the other hand, internal organs can also be affected in 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 butterfly-shaped on the face), painful and / or inflamed joints and muscles, and inflamed tendon sheaths (tendovaginitis). In addition, signs of inflammation of internal organs often develop (e.g. pleurisy, myocarditis, pericarditis, kidney inflammation).

You can read more about the diverse lupus symptoms associated with this form of disease in the article Systemic Lupus Erythematosus.

Lupus erythematosus: how dangerous is the disease?

According to the current state of knowledge, there is no cure for cutaneous lupus erythematosus. With the right therapy, including careful UV protection of the skin, the symptoms can usually be got under control.

The different forms of cutaneous lupus erythematosus can develop into systemic lupus with different frequencies. For example, in discoid lupus erythematosus (DLE) this happens in less than five percent of the cases, in contrast to subacute cutaneous lupus erythematosus (SCLE) in ten to 15 percent of the cases.

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 involved, SLE often takes a severe course. In individual cases, lupus can even be fatal. In Germany, however, most SLE patients have a normal life expectancy.

Lupus erythematosus: causes & risk factors

The exact causes of lupus erythematosus are not yet fully understood. According to experts, a genetic predisposition is the basis for the disorder of the immune system on which the disease is based. In combination with other factors, an outbreak of disease or a disease flare-up can occur:

First of all, UV light should be mentioned here. Other possible influencing factors are hormonal influences, since lupus erythematosus occurs much more frequently in women and girls than in men and boys (the hormonal balance in women is subject to greater fluctuations than in men). Other factors, such as stress and infections, can also trigger flare-ups.

Lupus erythematosus: examinations & diagnosis

At the beginning of the diagnosis of lupus erythematosus, the doctor will have a detailed discussion with the patient (in the case of children with the parents) about the medical history (anamnesis). He asks, for example, which symptoms exist, when they first appeared and whether or not what underlying diseases are known. This is followed by a physical examination, which is usually followed by further examinations.

Skin examination

Typical skin changes occur in the various forms of lupus disease. A lupus test at the dermatologist is therefore important for the diagnosis. To do this, the doctor takes a tissue sample (skin biopsy) from the affected areas of the skin. This is examined more closely in the laboratory using various methods.

Further investigations

In the case of cutaneous lupus erythematosus (CLE), standardized photo provocation can be useful in special cases. The skin is specifically exposed to UV light to check whether it reacts to it with typical CLE damage. These occur on average eight days (plus / minus 4.6 days) after UV exposure and then persist for a longer period of time. The photo provocation can help, for example, to differentiate a CLE from a polymorphic light dermatosis (UV-induced skin damage occurs earlier and then regresses).

Blood tests can also provide important clues 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.

In addition, if there is any suspicion of lupus disease, it must be clarified whether internal organs are also affected by the disease. If so, this suggests systemic lupus erythematosus. Helpful examinations can be, for example, imaging procedures (such as x-rays or ultrasound), an examination of the fundus or lung function tests.

You can read more about the extensive diagnostics of this form of lupus in the article Systemic Lupus Erythematosus.

Lupus erythematosus: treatment

Treatment for lupus erythematosus depends on the form and severity of the disease.

Cutaneous lupus erythematosus: therapy

The skin changes in skin lupus are specifically treated externally (local therapy). In some cases, patients must also take medication (systemic therapy). In addition, there are other measures that can have a positive effect on the course of the disease.

Local therapy

Using 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 calcineurin inhibitors: These are immunosuppressants, i.e. substances that have a depressant effect on the immune system (e.g. tecrolimus). They are recommended primarily for the local treatment of skin changes on the face and as an alternative to topical cortisone preparations.
Topical retinoids: Local treatment with these derivatives of vitamin A acid (such as tazarotene, tretinoin) is an option in severe cases of cutaneous lupus erythematosus.
Cold treatment, laser therapy: If other treatment measures against the skin changes do not help, cold treatment (cryotherapy) or laser therapy can be considered in selected cases.

Systemic therapy

It may be necessary to take additional medication, for example, if patients do not respond to other 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 drugs for skin lupus. Because of the risk of retinal damage, patients should have their eyes checked regularly by an ophthalmologist during treatment.
Glucocorticoids: The use of cortisone preparations should be limited in time because of the possible side effects. As soon as possible, it should be stopped by gradually reducing the dose (tapering off therapy).
Other immunosuppressants: In addition to cortisone, other immunosuppressants can also be given for skin lupus. Methotrexate (MTX), for example, is considered to be a second-choice drug in persistent cases of subacute cutaneous lupus (SCLE) and discoid lupus (DLE). If possible, it is used together with antimalarials. Other immunosuppressants for cutaneous lupus include azathioprine and ciclosporine.
Retinoids: In certain cases of skin lupus, it may be useful to take retinoids. They are also preferably used in combination with antimalarial agents.
Dapsone: This bacterial and anti-inflammatory agent can be prescribed, for example, to treat the bullous form of cutaneous lupus erythematosus.

In selected cases, a doctor can also prescribe other drugs to take, for example the strong anti-inflammatory drugs thalidomide or belimumab - a therapeutic antibody against certain immune cells.

Certain medications (e.g. retinoids) may not be used in patients who are currently pregnant or breastfeeding. The attending physician must take this into account when planning therapy.

A vitamin D supplement is prescribed for lupus patients when there is a vitamin D deficiency. Such is a risk factor for the development of cutaneous lupus erythematosus and systemic lupus erythematosus, among other things. If the deficiency is compensated, this can have a positive influence on the course of the disease in some cases.

Further measures

The 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 tanning salons) are just as unfavorable.

Refraining from active and passive smoking is also strongly advised. Nicotine consumption is a risk factor for cutaneous lupus erythematosus.

In some cases of subacute cutaneous lupus erythematosus (SCLE) it may be useful to avoid certain medications (in consultation with the treating doctor!). Some drugs can actually promote this form of lupus disease, for example the antifungal agent terbinafine, the dehydrating agent (diuretic) hydrochlorothiazide and various calcium channel blockers used to treat high blood pressure (such as verapamil).

Systemic lupus erythematosus: therapy

The treatment of systemic lupus erythematosus is more extensive because not only the skin but also internal organs are affected. Which these are and how severe the infestation is can vary from patient to patient. The treatment is therefore individually adapted.

You can find out more about this in the article Systemic Lupus Erythematosus.

Lupus erythematosus: prevention

The chronic inflammatory autoimmune disease lupus erythematosus cannot be prevented. However, one should avoid factors that cause the disease (with a corresponding genetic predisposition) to break out or trigger relapses in those who are already sick. In addition to stress and infections, this includes above all intense UV light (sun, artificial UV sources such as in solariums). You should avoid this even if you have an existing illness, because lupus erythematosus makes the skin more sensitive to sunlight.

In consultation with a doctor, taking vitamin D can also be a preventative measure.

Lupus erythematosus and diet

With a balanced diet you can alleviate some of the accompanying symptoms of systemic lupus erythematosus. Joint pain can be prevented by regularly adding fish to the menu.

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