Butterfly erythema

The Butterfly erythema is a symptom of a rare autoimmune disease, lupus erythematosus (PE), which occurs in two main forms. Cutaneous lupus erythematosus, one of the two main forms, occurs in many different subtypes and can also attack joints and internal organs as the disease progresses (systemic PE).

What is a butterfly erythema?

The doctors describe a symmetrical, inflammatory reddening of the face in the shape of a butterfly, which spreads from the bridge of the nose to both cheeks and the forehead, as butterfly erythema. It is often mistaken for a sunburn. The butterfly erythema is light red in color, flat or slightly raised, sharply defined and covered with scales. Those affected feel pain when touching the edges.

The skin changes sometimes go away on their own. However, if it is a symptom of lupus erythematosus (butterfly lichen), it turns into a scaly rash covered with blisters. It is restricted to the skin surface and the subcutaneous fat tissue.

The butterfly erythema can, however, also occur in connection with erysipelas (wound rose), a bacterial skin infection that affects the face and extremities. Lupus erythematosus is very rare. Out of 100,000 people only 12 to 50 fall ill, the age is usually between 15 and 25 years. The autoimmune inflammatory disease affects more women and girls than members of the male sex.

LE often occurs as a hybrid of different subtypes and is restricted to skin regions that are particularly exposed to UV light. Some subtypes of the cutaneous form, i.e. restricted to the skin, leave behind strongly sunken whitish scars with a brownish edge when they heal.

causes

In the majority of cases, butterfly erythema is a symptom of lupus erythematosus (PE), an autoimmune disease (collagenosis) that mainly affects young women between the ages of 15 and 25. It is still largely unknown why the immune system of those suffering from PE attacks the body's own connective tissue cells. Doctors assume that the disease is genetic.

Hormonal fluctuations and hormone changes (pregnancy, pill), certain medications, viral diseases, tiny skin injuries, strong sunlight and psychological or physical stress are also discussed as causes. It is characteristic that the butterfly erythema often appears after intensive sunbathing (vacation in southern countries) and during or after pregnancy.

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Diseases with this symptom

• Systemic lupus erythematosus (SLE)

• Hormonal disorders (hormonal fluctuations)

• Sore rose

Diagnosis & course

After taking the anamnesis, the patient is thoroughly examined physically. At the dermatologist's, he has to undergo a skin examination in which several biopsies are made to find out the exact cause of the skin change. His blood is tested for auto-antibodies, which are always present in systemic lupus erythematosus and in most cases in subacute cutaneous PE.

These are antinuclear factors (ANA), ds-DNA antibodies, etc. Then, through further examinations, the doctor can find out whether internal organs are affected by the disease.Since lupus erythematosus is an extremely rare disease, medicine currently does not have any laboratory values ​​with which the disease activity can be reliably assessed. In two thirds of patients, it takes place in phases and is initially limited to the surface of the skin.

In one third of those affected, PE has a chronic, insidious course. There can be years without symptoms between the flare-ups. Sometimes the inflammatory skin changes turn into systemic lupus erythematosus (SLE). In his case, the joints and various organs are also pathologically changed. This is the case in almost all patients with acute cutaneous PE (ACLE).

In subacute cutaneous PE (SCLE) this course occurs in only ten to fifteen percent of patients, in chronic discoid PE (CDLE) in a maximum of five percent of cases. The other lupus subtypes are only limited to the skin. Patients with CDLE have a good chance that their disease will come to a standstill after a few years or decades at the latest.

Subacute cutaneous lupus erythematosus also has a favorable prognosis. If the lungs, heart and kidneys are affected in systemic lupus erythematosus, serious illnesses and occasionally even deaths can occur.

Complications

A butterfly erythema occurs particularly in systemic lupus erythematosus (SLE), which has numerous complications. This autoimmune disease can affect any organs in the body, especially the kidneys and the central nervous system. In the kidneys, lupus erythematosus leads to an inflammation known as lupus nephritis. If left untreated, this can quickly turn into kidney failure (renal insufficiency).

In the long term, this leads to increased blood pressure (hypertension). In addition, there are disturbances in the acid-base balance and electrolyte balance. The result is a reduced excretion of acids by the kidneys, the pH of the blood rises and acidosis develops. This acidosis leads to hyperkalemia, i.e. an increased potassium level in the blood, which can lead to severe cardiac arrhythmias which can lead to cardiac arrest.

In addition, the kidneys can no longer excrete enough fluid, so that it accumulates in the body and edema develops. Finally, kidney failure can lead to anemia and vitamin D deficiency. The involvement of the central nervous system can be accompanied by inflammation of the brain or the spinal cord (encephalitis or myelitis).

This can lead to paralysis or epileptic seizures. In the worst cases, it can also lead to paraplegia in the patient. Blindness is also conceivable. In addition, those affected are more susceptible to infections and malignant diseases.

When should you go to the doctor?

In many cases, the symptoms or symptoms of butterfly erythema are inconsistent, so that an early diagnosis of this disease is not possible in many cases. However, you should always consult a doctor immediately if you have kidney problems or if the central nervous system is restricted. In the worst case scenario, without treatment it can lead to paralysis or complete kidney failure. A doctor must also treat you if you have heart problems. The affected person can die without treatment.

A doctor should also always be consulted in the event of epileptic seizures. In the further course, the butterfly erythema can also lead to complete paraplegia. As a rule, this cannot be treated by a doctor. However, an examination is still useful, as it can be used to carry out certain therapies that may lead to an improvement in this disease. Rashes that cannot be linked to any other complaint can also be a symptom of butterfly erythema and should be examined by a doctor.

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Treatment & Therapy

The butterfly erythema, which only occurs on the face, and the discoid rashes that also occur in other parts of the body, respond well to treatment with cortisone ointment. In severe cases, the doctor will administer immunosuppressants such as ciclosporin A or azathioprine and cytostatics, which are supposed to inhibit cell growth. Patients are advised not to expose themselves to direct sun and always to apply sun blockers with a high sun protection factor so that their condition does not worsen.

If the skin changes do not recede or if other areas of the skin exposed to light are affected by the skin inflammation, the doctor uses an antimalarial drug that contains hydroxychloroquine or chloroquine. Treatment with this agent is successful in three out of four cases. It is accompanied by regular examinations of the fundus and blood tests.

In addition to the antimalarial agent, cortisone tablets or cortisone infusions are also given in the case of pronounced butterfly lichen. If the patient suffers from systemic lupus erythematosus, the individual diseases must also be treated: For arthritis, he is given non-steroidal anti-inflammatory drugs (NSAIDs) and painkillers. Sometimes the administration of belimumab, a monoclonal antibody, is also useful.

Outlook & forecast

As a rule, there is a strong reddening of the face with a butterfly erythema. The redness itself is also associated with pain that occurs when touched.

Many sufferers feel disfigured and unattractive by the butterfly erythema, which has a negative effect on the patient's self-esteem and can also lead to psychological problems. In some cases, blisters can appear on the rash. The quality of life is thus greatly reduced by the butterfly erythema.

In most cases, butterfly erythema can be treated with the help of creams and ointments. If these do not help, immunosuppressants are given. The patient is also no longer allowed to stay in the sun for long without sun protection and is therefore restricted in his activities.

If the butterfly erythema does not go away on its own, medication against malaria can be used, which are usually very effective against the symptoms. In severe cases, tablets containing cortisone can be taken. The pain is treated with pain relievers.

In most cases, the butterfly erythema can be restricted or completely cured. The symptom does not reduce life expectancy.

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prevention

Prevention is not yet possible as the exact causes of the inflammatory disease are not yet known. If the genetic predisposition is known, however, the person affected should try to avoid the triggers (infections, stress, strong sunlight).

You can do that yourself

It is particularly important to protect sensitive skin from exposure to the sun. Direct contact with the sun should be avoided as far as possible or at least reduced. It is just as important to use sunscreen with a very high sun protection factor and to wear closed clothing and a hat. These precautionary measures must also be observed in winter. Protecting the skin from the cold is just as important as it is from the heat. On excursions, care should be taken to ensure that there is sufficient shade.

Nicotine also has a negative effect on the course of the disease. Those affected should stop smoking if possible and avoid closed rooms in which people smoke. The intake of estrogen preparations has an equally unfavorable effect on the disease. An alternative method of contraception should be sought with the specialist. Strengthening the immune system is also important. For this, the consumption of a diet rich in vital substances, exercise in the fresh air and relaxation phases are decisive. Since the butterfly erythema is an inflammatory process, the body can be supported by taking micronutrients such as selenium. These work actively against the inflammation process in the body.

In the treatment of butterfly erythema, the increased consumption of omega-3-containing foods such as fatty sea fish (salmon, cod, mackerel) or linseed oil has proven itself. These should be incorporated into the menu as often as possible.