Butterfly Erythema: Causes, Treatment & Help

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

What is butterfly erythema?

The medical profession refers to butterfly erythema as a symmetrical inflammatory redness of the face in the shape of a butterfly, spreading from the bridge of the nose to both cheeks and the forehead. It is often mistaken for sunburn. Butterfly erythema is bright red in color, flat or slightly raised, sharply demarcated, and covered with scales. When touching the edges, affected individuals feel pain. The skin change sometimes disappears on its own. However, if it is a symptom of lupus erythematosus (butterfly rash), it turns into a scaly rash covered with vesicles. It is confined to the skin surface and subcutaneous fatty tissue. However, butterfly erythema may also occur in association with erysipelas (erysipelas), a bacterial skin infection that affects the face and extremities. Lupus erythematosus is very rare. Only 12 to 50 people out of 100,000 develop the disease, and the age is usually between 15 to 25 years. The inflammatory autoimmune disease affects more women and girls than members of the male sex. LE often occurs as a mixed form of different subtypes and is restricted to skin regions that are particularly exposed to UV light. Some subtypes of the cutaneous, or skin-restricted, form leave severely sunken whitish scars with brownish margins as they heal.

Causes

In the majority of cases, butterfly erythema is a symptom of lupus erythematosus (LE), an autoimmune disease (collagenosis) that primarily affects young women between the ages of 15 and 25. Why it is that the immune system of those suffering from LE attacks the body’s own connective tissue cells is still largely unknown. Doctors assume that the disease is genetically determined. Hormonal fluctuations and hormone changes (pregnancy, contraceptive pill), certain medications, viral diseases, tiny skin injuries, strong sunlight and psychological or physical stress are also discussed as causes. Characteristically, butterfly erythema often appears after intense sunbathing (vacations in southern countries) and during or after pregnancy.

Diseases with this symptom

  • Systemic lupus erythematosus (SLE).
  • Hormonal disorders (hormonal fluctuations)
  • Erysipelas

Diagnosis and course

After taking a medical history, the patient undergoes a thorough physical examination. At the dermatologist’s office, he must undergo a skin examination, during which several biopsies are taken to determine the exact cause of the skin change. His blood is tested for autoantibodies, which are always present in systemic lupus erythematosus and are present in most cases of subacute cutaneous LE. These are antinuclear factors (ANA), ds-DNA antibodies, etc. Then the physician finds out by further examinations whether internal organs are affected by the disease. Since lupus erythematosus is an extremely rare disease, medical science does not yet have laboratory values that can be used to reliably assess disease activity. It progresses in relapses in two-thirds of patients and is initially confined to the skin surface. In one third of those affected, LE has a chronic, gradual course. There may be years between episodes of the disease without symptoms. Sometimes the inflammatory skin changes turn into systemic lupus erythematosus (SLE). In this case, the joints and various organs are also pathologically altered. This is the case in almost all patients with acute cutaneous LE (ACLE). In subacute cutaneous LE (SCLE), this course occurs in only ten to fifteen percent of patients, and in chronic discoid LE (CDLE) in a maximum of five percent of cases. The other lupus subtypes are limited to the skin only. Patients with CDLE have a good chance that their disease will arrest after several years to 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, severe disease and, in isolated cases, even death can result.

Complications

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 kidney and central nervous system. In the kidney, lupus erythematosus leads to an inflammation called 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 of the acid-base balance and electrolyte balance. The result is a reduced excretion of acids via the kidneys, the pH of the blood rises and an acidosis develops. This acidosis leads to hyperkalemia, i.e. elevated potassium levels in the blood, which can result in severe cardiac arrhythmias that can lead to cardiac arrest. In addition, the kidneys can no longer excrete enough fluid, so that increased fluid accumulates in the body, resulting in edema. Finally, anemia and vitamin D deficiency may occur due to renal insufficiency. Infestation of the central nervous system may be accompanied by inflammation of the brain or spinal cord (encephalitis or myelitis, respectively). This can lead to paralysis or epileptic seizures. In the worst cases, it can also lead to paraplegia of the patient. Blindness is also conceivable. In addition, affected individuals are more susceptible to infections and malignant diseases.

When should you see a doctor?

In many cases, butterfly erythema does not present with uniform complaints or symptoms, so early diagnosis of this disease is not possible in many cases. However, a doctor should always be consulted immediately if kidney symptoms or central nervous system limitations are present. Without treatment, paralysis or complete kidney failure can occur in the worst case. Treatment by a doctor is also required for heart complaints. In this case, the affected person may die without treatment. A doctor must also always be consulted in the event of epileptic seizures. In the further course, butterfly erythema can also lead to complete paraplegia. This usually cannot be treated by a physician. However, an examination is still useful, as it can be used to carry out certain therapies that may lead to an improvement of this disease. Skin rashes that cannot be associated with any other complaint may also be a symptom of butterfly erythema and should be examined by a physician.

Treatment and therapy

Butterfly erythema, which occurs only on the face, and discoid rashes, which occur elsewhere on the body, respond well to treatment with cortisone ointment. In severe cases, the physician administers immunosuppressants such as ciclosporin A or azathioprine and cytostatics to inhibit cell growth. Patients are urged not to expose themselves to direct sunlight and to always apply sunblock with a high sun protection factor so that their disease 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 physician uses an antimalarial drug containing hydroxychloroquine or chloroquine. In three out of four cases, treatment with this drug is successful. It is accompanied by regular examination of the back of the eye and checks on blood values. In cases of severe butterfly rash, cortisone tablets or cortisone infusions are administered in addition to the antimalarial agent. If the patient suffers from systemic lupus erythematosus, the individual diseases must also be treated: In the case of arthritis, he receives non-steroidal anti-inflammatory drugs (NSAIDs) and painkillers. Sometimes the administration of belimumab, a monoclonal antibody, is also useful.

Outlook and prognosis

Usually, butterfly erythema results in severe redness of the face. The redness itself is also associated with pain when touched. Many affected individuals feel disfigured and unattractive as a result of butterfly erythema, which has a negative effect on the patient’s self-esteem and can also lead to psychological problems.In some cases, vesicles may also appear on the rash. Thus, the quality of life is greatly reduced by butterfly erythema. In most cases, butterfly erythema can be treated with the help of creams and ointments. If these do not help, immunosuppressants are administered. The patient is also not allowed to spend long periods of time in the sun without sunscreen and is thus restricted in his or her activities. If the butterfly erythema does not disappear on its own, anti-malarial drugs can be used, which are usually very effective against the symptoms. In severe cases, tablets with cortisone can be taken. The pain is treated with painkillers. Butterfly erythema can be limited or completely cured in most cases. Life expectancy is not reduced by the symptom.

Prevention

Prevention is not currently possible because the exact causes of the inflammatory disease are not yet known. However, if a genetic predisposition is known, the affected person should try to avoid the triggers (infections, stress, strong sunlight).

What you can do yourself

It is particularly important to protect sensitive skin from sunlight. Direct contact with the sun should be avoided as far as possible or at least reduced. Equally important are the use of a sunscreen with a very high sun protection factor and the wearing of closed clothing and a head covering. These precautions should also be observed in winter. Protecting the skin from cold is just as important as protecting it from 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. Patients should stop smoking if possible and avoid closed rooms where people smoke. Taking estrogen preparations has an equally unfavorable effect on the disease. Here, an alternative contraceptive method should be sought with the specialist. It is also important to strengthen the immune system. For this, the intake of a diet rich in vital substances, exercise in the fresh air and relaxation phases are crucial. Since butterfly erythema is an inflammatory process, the body can be supported by taking micronutrients – such as selenium. These actively work against the inflammatory process in the body. In the treatment of butterfly erythema, the increased intake of omega-3-containing foods, such as fatty sea fish (salmon, cod, mackerel) or linseed oil, has also proven effective. These should be incorporated into the diet as often as possible.