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Lupus erythematosus: causes, forms, therapy

by Josephine Andrews
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Lupus erythematosus (butterfly lichen, butterfly erythema) is an autoimmune disease that occurs primarily in women. The immune system mistakenly attacks the body’s own cell structures. In most cases, the course of the disease is more or less limited to the skin, as in the case of cutaneous lupus erythematosus. However, internal organs can also be affected (systemic lupus erythematosus). Read more about the causes, symptoms, diagnosis and treatment of lupus disease here.

quick overview

  • What is lupus erythematosus? Rare chronic inflammatory autoimmune disease that primarily 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. kidney inflammation , joint pain ).  
  • How dangerous is the disease? Cutaneous lupus has a good prognosis (although there is no cure). Sometimes it progresses to systemic lupus. Life expectancy is then usually also normal. Fatal outcomes are possible, however.  
  • Causes & risk factors : The presumed cause is a disorder of the immune system. Factors such as UV light , medication, hormonal changes, stress and infections can promote the disease or trigger flare-ups.
  • Examinations : Interview, skin and blood tests. If SLE is suspected, internal organs should also be 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 collagen diseases. These are connective tissue diseases that belong to the inflammatory rheumatic diseases.

In 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, physicians 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, more often in women than in men. Data on the prevalence of the disease in the population are limited. A Swedish study found four new cases of CLE per 100,000 inhabitants.

CLE usually 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 subtypes, the most common being discoid lupus erythematosus (DLE) .
  • Intermittent cutaneous lupus erythematosus (ICLE) – with a subtype

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

Systemic lupus erythematosus (SLE)

In addition to the skin, this type of lupus also affects a wide variety of internal organs. Inflammations in the kidneys, lungs and heart are common, for example . 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 young adult women. However, the disease can also break out in childhood.

You can read more about this form of lupus disease in the article Systemic lupus erythematosus .

Lupus erythematosus: frequency

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

Lupus erythematosus: symptoms

Which symptoms occur in lupus erythematosus depends crucially on the course of the disease. The disease can be limited to the skin, but can also affect internal organs.

Discoid lupus erythematosus (DLE)

Typically, the appearance of disc-shaped, sharply demarcated, slightly raised erythema that is surrounded by rough scales indicates a flare-up in this form of lupus. The parts of the body that are often exposed to the sun, such as the nose , forehead, cheeks, lips, auricles and the backs of the hands , are particularly affected . On the face, the skin reddening often looks butterfly-shaped . Therefore, lupus erythematosus is also called butterfly disease.

The reddish-scaly skin lesions spread outwards, while they slowly heal from the center with the scales detaching. A horny plug can be seen on the underside of the detached scales. This so-called “ paper nail phenomenon” is typical of discoid lupus erythematosus. The skin under the shed scales is thin, shiny, white and – on the hairy head – hairless.

Common lupus symptoms in the discoid form of the disease are also dimpled scars with visibly dilated small skin vessels (telangiectasia) and patchy skin areas 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 being 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 in discoid lupus and sometimes resemble psoriasis . Scarring and pigment disorders rarely occur.

On the other hand, subacute cutaneous lupus erythematosus can also affect internal organs 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 (tenovaginitis). In addition, there are often signs of inflammation of internal organs (e.g. pleurisy , myocarditis , pericarditis , nephritis).

You can read more about the diverse lupus symptoms associated with this form of the disease in the article on systemic lupus erythematosus .

Lupus erythematosus: how dangerous is the disease?

According to the current state of knowledge, cutaneous lupus erythematosus cannot be cured. With the right therapy, including careful UV protection of the skin, the symptoms can usually be brought under control.

The various forms of cutaneous lupus erythematosus can progress to systemic lupus with varying frequency. For example, this occurs in less than 5 percent of discoid lupus erythematosus (DLE) cases, but in 10 to 15 percent of subacute cutaneous lupus erythematosus (SCLE) 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 some 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 immune system disorder on which the disease is based. In combination with other factors , the disease can then break out or flare up:

First and foremost is UV light. Other possible influencing factors are hormonal influences, since lupus erythematosus occurs much more frequently in women and girls than in men and boys (the hormone 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 lupus erythematosus diagnosis, the doctor will have a detailed discussion with the patient (in the case of children with the parents) about the medical history (anamnesis). He inquires, for example, which symptoms exist, when they first appeared and whether any 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 by a dermatologist is therefore important for the diagnosis. To do this, the doctor takes a tissue sample ( skin biopsy ) from the affected skin areas . This is examined more closely in the laboratory using various methods.

Further investigations

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

Blood tests can also provide important information about the autoimmune disease. For example, specific antibodies can be detected in the blood in systemic lupus erythematosus and in most cases of subacute cutaneous lupus erythematosus.

In addition, whenever a lupus disease is suspected, 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

The treatment of 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 have to take additional medication ( systemic therapy ). There are also other measures that can have a positive effect on the course of the disease.

local therapy

Using a local (topical) therapy, the inflammatory skin changes in cutaneous lupus erythematosus are treated from the outside in a targeted manner:

  • Topical glucocorticoids (“cortisone”) : Circumscribed areas with skin changes are preferably treated with topical cortisone preparations (eg cortisone ointment). The application should be as short as possible due to the possible side effects.
  • Topical calcineurin inhibitors : These are immunosuppressants, ie substances that have a dampening 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 topical cortisone preparations.
  • Topical retinoids : Topical 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

Additional medication may be necessary, for example, if patients do not respond to other medications or if the skin damage is very severe. The following groups of active ingredients are available:

  • Antimalarials : Active ingredients such as chloroquine or hydroxychloroquine are among the most important basic medications for skin lupus. Because of the risk of retinal damage, patients should have their eyes checked regularly by an ophthalmologist during treatment.
  • Glucocorticoids : The intake of cortisone preparations should be limited in time because of the possible side effects. As soon as possible, it should be ended by gradually reducing the dose (tapering off the therapy).
  • other immunosuppressants : In addition to cortisone, other immunosuppressants can also be given for skin lupus. For example, methotrexate (MTX) is considered a second-line drug in intractable cases of subacute cutaneous lupus (SCLE) and discoid lupus (DLE). Whenever possible, it is used in conjunction with antimalarials. Other immunosuppressants used in cutaneous lupus include azathioprine and cyclosporine .
  • Retinoids : In certain cases of skin lupus, taking retinoids can be useful. They are also preferably used in combination with antimalarials.
  • Dapsone : This antibacterial 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 be taken, for example the powerful anti-inflammatory drug thalidomide or belimumab – a therapeutic antibody against certain immune cells.

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

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

Further measures

Consistent sun protection is part of the treatment of cutaneous lupus erythematosus : patients should avoid direct sunlight and use sunscreen 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 considered a risk factor for cutaneous lupus erythematosus.

In some cases of subacute cutaneous lupus erythematosus (SCLE), avoiding certain medications can make sense (in consultation with the doctor treating you!). Some drugs can promote this form of lupus disease, such as the antifungal drug terbinafine , the water pill (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. What these are and how severe the infestation is can vary from patient to patient. Accordingly, the treatment is individually adapted.

You can read 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 can cause the disease (if there is a genetic predisposition) to break out or trigger relapses in those who are already ill. In addition to stress and infections, this includes, above all, intensive UV light (sun, artificial UV sources such as in solariums). You should also avoid these if you have an existing 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 .

Lupus erythematosus and diet

A balanced diet can alleviate some of the accompanying symptoms of systemic lupus erythematosus . Joint pain can be prevented by regularly including fish on the menu.

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