Introduction to Dermatomyositis

In the case of poor general well-being with sudden muscle weakness or persistent muscle soreness, especially in women and children, dermatomyositis or purple disease should be considered. Certain skin changes on the face and neck are also signs of this disease.

What is dermatomyositis?

According to abbreviationfinder, dermatomyositis is a rheumatic disease in which there is an inflammatory reaction of the skin and the striated muscles. It is a rare, phased disease that can occur in both childhood and adulthood.

The juvenile form begins there in old agefrom seven to eight years. Both sexes are affected equally often. In contrast, the adult form of dermatomyositis is far more common in women between the ages of 35 and 44 and between 55 and 60 years. From the age of 50, in addition to rheumatic muscle disease, tumors in the gastrointestinal area, in the chest and in the lungs occur more frequently.

Causes

The cause of the development of dermatomyositis has not yet been determined. However, it is suspected that certain autoimmune diseases are involved in the development process, in which the immune defense incorrectly targets the body’s own muscle and skin cells instead of attacking invading bacteria or viruses as usual.

Those affected therefore complain of general muscle weakness and fatigue, and sometimes also of hair loss. Everyday activities can only be carried out with difficulty. The skin changes occur primarily as swelling of the eyelids and reddening of the skin on the face and neck.

The nail fold on the hands is often painfully inflamed and discoloration of the skin as well as areas of skin that are noticeable due to their different skin thicknesses develop. In dermatomyositis, this mainly affects parts of the body that are exposed to light.

Symptoms, ailments & signs

Dermatomyositis can lead to very severe restrictions in the life and everyday life of the person affected and thereby also significantly reduce the patient’s quality of life. First and foremost, those affected by this disease suffer from severe muscle weakness. It is therefore not uncommon for the patient to be permanently tired and exhausted, so that most of those affected no longer actively participate in everyday life.

It is also no longer possible to carry out strenuous physical activities or sports without further ado. Furthermore, there is severe pain in the muscles, even if they have not been stressed too much. In dermatomyositis, the pain resembles sore muscles and significantly restricts the patient’s everyday life.

This can also lead to restricted mobility, so that some of those affected are dependent on the help of other people in their lives. Discomfort can also occur on the face, so that some people suffer from swelling or reddening of the skin. The symptoms of dermatomyositis often lead to psychological complaints, with some patients suffering from depression or other psychological disorders. Whether dermatomyositis leads to a reduced life expectancy depends very much on the exact cause of the disease.

Diagnosis

The diagnosis of dermatomyositis is often already made based on the muscle complaints and changes in the patient’s skin and confirmed by a laboratory blood test.

An increased creatinine kinase value (CK) and increased inflammation parameters are characteristic of this disease. Autoantibodies can also be detected in the blood analysis. To detect irregularities in the muscle fibers and swellings, the muscles are assessed during an ultrasound examination.

Magnetic resonance imaging (MRI) is sometimes useful for diagnosis, as this imaging method can show possible muscle changes particularly early. In order to rule out other diseases and thus to secure the diagnosis of dermatomyositis, a skin sample or a muscle biopsy is carried out under local anesthesia. The tissue obtained can then be examined histologically.

Complications

Dermatomyositis leads to a general feeling of illness and weakness. The patient is no longer able to perform physical activities and is therefore severely restricted in his everyday life. Muscle strength also decreases and is usually accompanied by changes in the skin.

Those affected suffer from permanent fatigue. This tiredness is not combated by the patient resting for a long time or sleeping longer. After physical activity there is severe muscle soreness, which in some cases can also occur without activity.

The pain usually results in restrictions in movement and thus in everyday life. The face suffers from swelling and reddening of the skin, so that the patient usually feels uncomfortable and ashamed of these symptoms. It is not possible to treat dermatomyositis directly.

However, immunosuppressants are used to prevent the body from reacting. The patient also needs a sufficient supply of vitamins and trace elements. As a rule, it is not advisable for those affected to spend long periods in the sun without sun protection.

The muscle complaints can be treated with physiotherapy. There are no further complications. However, with permanent dermatomyositis, the patient is severely restricted in his everyday life.

When should you go to the doctor?

If you notice swelling and reddening of the skin on your face and neck, you may have dermatomyositis. A visit to the dermatologist is recommended if the skin changes persist longer than usual and cannot be traced back to a clear cause. Risk groups such as people with autoimmune diseases or an existing skin disease should speak to their doctor about the symptoms mentioned. If the complaints have a massive impact on well-being, a medical clarification is necessary in any case.

Symptoms such as muscle weakness, tiredness or restricted mobility need to be clarified immediately. Anyone who already notices complications such as increasing mobility restrictions or intense fatigue should consult a doctor immediately and have the cause clarified. Once diagnosed, dermatomyositis can be treated well.

If left untreated, however, further complaints can arise that massively reduce well-being. Therefore: in the event of unusual skin changes and muscle complaints, inform the doctor directly. In addition, a physiotherapeutic treatment is recommended. The measures of physiotherapy alleviate the acute symptoms and prevent a new illness.

Treatment & Therapy

Dermatomyositis is generally not curable. The treatment of the disease is therefore based on the symptoms of the individual patient and their age. The first step is to intervene in the disease process with immunosuppressants and anti-inflammatory drugs in order to suppress the inflammatory reaction and weaken the immune system ‘s reaction to the body’s own cells.

The anti-inflammatory preparations must initially be taken in high doses, but can be reduced after a while as they cause numerous side effects. As the disease progresses, the dosage must be adjusted more frequently to the acute symptoms of the patient. However, an immunosuppressive drug must be taken permanently to relieve the symptoms.

The anti-inflammatory preparations can lead to osteoporosis. As a preventive measure, the patient must ensure that there is sufficient intake of vitamin D and calcium. Since this is hardly possible through food, appropriate medication is prescribed. The diet should also be low in salt.

Due to the skin changes, sufficient sun protection should also be ensured. Dermatomyositis is phased. In the event of an acute flare-up of illness, the patient must remain in bed, but after the symptoms have subsided, they should help maintain their muscle functions with physiotherapy and other physiotherapeutic applications.

Outlook & forecast

Dermatomyositis initially has a chronic course that cannot be stopped. The disease cannot be cured with current medical options. The treatment plan provides relief from the symptoms that are present. In addition, the progress of the disease should be delayed as much as possible. In long-term therapy, the rheumatic complaints can be alleviated and the quality of life is considerably improved.

The peculiarity of the disease becomes apparent after a few years. After five to ten years, the disease progresses to a standstill in over 80% of patients. The reasons lie in the therapeutic measures taken or in an inexplicable spontaneous death of the disease. The remaining 20% ​​of sufferers continue to suffer from dermatomyositis after more than ten years and do not experience any recovery. You belong to a risk group that increasingly suffers from sequelae.

The prognosis for dermatomyositis worsens as soon as complications arise. If a tumor develops, the patient’s condition can be life-threatening. In a large number of sufferers, a malignant tumor forms in the heart or lungs. Just over 60% of the sick survive the first five years after the diagnosis. After ten years it is almost half of all those affected.

Prevention

The development of dermatomyositis cannot be actively prevented. After the diagnosis has been made, however, the patient should definitely take advantage of physiotherapeutic applications between the acute phases of the disease to maintain his muscle functions in order to counteract permanent restriction of movement.

Aftercare

In most cases, follow-up care options for dermatomyositis are severely limited. The patient is primarily dependent on the correct examination, diagnosis and treatment of this disease, so that further compilations and complaints are prevented. In any case, early treatment has a positive effect on the further course and should therefore always be aimed for.

In most cases, patients with dermatomyositis depend on the use of immunosuppressive drugs. These should therefore be taken regularly and as directed by a doctor. In cases of doubt or other uncertainties, a pharmacist or doctor should always be consulted in order to avoid further discrepancies.

Many patients are also dependent on taking supplements with vitamin D, although a healthy lifestyle with a balanced diet can also have a positive effect on the course of dermatomyositis. In general, the person concerned should keep bed rest and therefore not exert themselves unnecessarily or move unnecessarily. Stressful situations should therefore also be avoided as far as possible. Physiotherapy rehabilitation measures are necessary in some cases, although some of the exercises can also be performed in your own home.

You can do that yourself

Sunlight or artificial UV light can trigger dermatomyositis or worsen an existing disease. Patients should therefore avoid extensive sunbathing, avoid being outdoors during the midday heat in the summer months, if possible, and always use a sunscreen with a sun protection factor of 25 or higher. Solarium visits are also not recommended during acute dermatomyositis.

The medically prescribed cortisone preparations should be used consistently, despite the side effects. Under no circumstances should cortisone therapy simply be stopped without consulting a doctor. Since cortisone promotes the development of osteoporosis, those affected can take countermeasures here. It makes sense to take calcium and an adequate supply of vitamin D.

For this purpose, calcium-rich foods can be consumed, although the intake of calcium from cow’s milk is now highly controversial. Plant-based milk alternatives fortified with calcium are preferable here. Calcium can also be purchased in the form of effervescent or chewable tablets in pharmacies or health food stores. The body produces vitamin D itself, provided that there is sufficient sunlight available. Since UV rays have a harmful effect on dermatomyositis, dietary supplements should also be used here.

The purple spots on the skin can be covered with a green concealer or, in severe cases, with special camouflage make-up.

Dermatomyositis