If you or your loved ones have dermatomyositis, be sure to read on:
What is dermatomyositis?
What are the clinical signs and manifestations of dermatomyositis?
In this article, we will introduce you to dermatomyositis as a common inflammatory disorder.
A person with dermatomyositis develops certain skin manifestations in addition to muscle weakness, which intensifies at certain times and decreases at times, making the patient asymptomatic.
Dermatomyositis consists of a combination of the dermis (skin) and the myo (muscle) itis (a suffix used in medicine to mean inflammation).
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Dermatomyositis and statistics and epidemiology
Statistics show that out of every one million people in the world, 7/7-9/1% (in some references two to ten people per million) suffer from dermatomyositis and it has a prevalence of about one in ten thousand to one in fifty thousand people.
In whom is dermatomyositis more common?
The onset of dermatomyositis is usually in adulthood. Some people develop dermatomyositis at a younger age, which is classified as juvenile dermamyositis.
All races have the same susceptibility to dermatomyositis
Women are three times more likely than men to develop dermatomyositis (in some references even twice).
Some inherent and genetic traits are predisposing to the disease:
HLA B8 / DR3
Although the cause of dermatomyositis is not fully understood, some viruses may be involved.
Such as viruses:
rubella virus
Flu
Coxsackie
The highest prevalence of dermatomyositis in children is five to fifteen years old
Another peak is in the late forties to early sixties.
Why does dermatomyositis occur?
What causes dermatomyositis?
The exact cause of dermatomyositis is not yet known. Doctors consider this disorder to be a mistake of the immune system of the affected person.
That is, the immune system of a person with a mistake against the muscle cells and skin of the person enters the attack and fights and damages them. These diseases are called autoimmune disorders.
It is said that the very thin walls of the arteries in the muscles are probably the first parts of the immune system to be mistaken for foreign tissue and become inflamed.
What are the symptoms of dermatomyositis?
- Skin manifestations in dermatomyositis:
It is the first clinical and physical manifestation of the disease and appears one year before the onset of other symptoms.
Skin lesions are purple or red in color and appear on the face, eyelids, chest, back, ankles, elbows and knees.
These lesions are sometimes itchy and can sometimes be painful. Skin lesions usually occur in areas of the skin surface that are sun exposed.
In these areas, the lesions are often itchy or painful and are known as scarves because they appear on the back of the shoulders and neck exactly where you put on your scarf.
Red to purple lesions that form on the eyelids and are swollen are called heliotrope lesions. Red to purple rashes also appear on the elbows and knees and sometimes on the fingers, especially the toes, which doctors call Gottron’spapules.
- There are changes in the nails:
Nail capillaries will undergo changes that your doctor will see with a capiloroscope, a microscopic-sharp instrument.
Calcium will deposit in the skin and later in the muscles, we see more calcinosis in children.
Calcium deposits in the shoulders, legs, thighs, and pelvis are said to form years after the onset of the disease and sometimes cause movement problems in the affected person.
- Muscular manifestations in dermatomyositis:
Weak muscles in the buttocks and thighs, shoulders and arms, as well as the neck will severely reduce the strength of the muscles in these areas.
Muscles are involved on both sides of the body and are highly progressive and will become more intense over time.
Other symptoms:
- Redness around fingernails
- Inflamed and dry skin with scaling
- Thinning hair
- Hard bumps under the skin, which are actually calcium deposits or calcinosis
- Difficult to swallow
- Change voice
- Fever, weight loss and extreme tiredness
- Muscle aches
- Weak muscles in the pelvis and upper thighs that make it difficult to get up from a sitting position
Sometimes inflammation and weakness spread to the gastrointestinal tract, heart and lungs
In adults, lung involvement following inflammation is associated with mild fever and cough.
diagnosis
History will be the basis of diagnosis in addition to observation and examination of clinical manifestations as well as physical examination. The following tests are used to confirm the main diagnosis and reject other differential diagnoses.
- blood test:
Measurement of muscle enzymes such as:
- Creatine kinase or CK
- Aldolase
- LDH
Increased creatine kinase and aldolase will be signs of inflammation and muscle damage.
Specific antibodies that accompany the onset of skin symptoms are also sometimes requested
ESR is increased in 50% of patients
The important point is that in elderly men with the disease, the necessary tests must be performed to find the possible cancer that has predisposed to the manifestation of dermatomyositis.
- EMG_NCV or electromyography:
With this test, the doctor will measure how the motor muscles function
If the pattern of muscle contraction changes, it could indicate myositis. This electrograph determines which muscles will be involved.
- MRI:
Shows the amount of inflammation in the muscles.
- Sampling or biopsy of skin lesions or weakened muscles:
Doctors say that if a sample taken from a skin lesion confirms a disease, it will no longer be necessary to sample the muscle.
treatment
- Rest:
In the onset phase of symptoms, you should rest, and during this period, standard exercises should be performed to prevent weight loss and muscle weakness.
- Physiotherapy:
It contains muscle strengthening exercises and is very effective in maintaining the normal range of motion of the joints.
- Corticosteroids:
The first line of drug treatment for dermatomyositis is corticosteroids.
With the start of these medications, the sufferer will recover greatly within the first few weeks. It takes three to six months to see improvement in some cases. It is said that this disorder will go away in fifty to seventy percent of all cases with this treatment.
- Immunosuppressants:
In a group of cases that do not respond well to corticosteroids, your doctor may prescribe immunosuppressive drugs.
- Methotrexate
- Azathioprine
- Cyclosporine
- Mycophennlate Mofetil
These drugs are considered to have the onset of positive effects in two to six months. In other words, these drugs cannot be used to control symptoms immediately.
With the start of using these drugs, the dose of corticosteroids is reduced. Cyclophosphamide will be used to treat and control lung involvement. If you are pregnant or planning to become pregnant, you should tell your doctor.
Be sure to get enough information about the side effects of these drugs.
- Hydroxychloroquine:
It will be effective in treating and controlling skin lesions but has no role in controlling muscle inflammation
- Intravenous immunoglobulins or IVIG:
- Rituximab:
In cases resistant to drug therapy and intravenous injection of immunoglobulin
People with dermatomyositis are strongly advised to avoid exposure to sunlight.
Complications of dermatomyositis
- Problems swallowing:
Difficulty swallowing can lead to decreased food intake and weight loss.
- Pneumonia and lung infections:
Difficulty swallowing and dysfunction of the pharyngeal muscles lead to the entry of contaminated saliva and food into the lungs, which leads to aspiration pneumonia or infection of the lung tissue following aspiration. Respiratory involvement is manifested by coughing and shortness of breath.
It is said that people with dermatomyositis are more likely than others in the community to develop the following diseases and disorders:
- Having other autoimmune disorders:
- Lupus
- Scleroderma
- Sjogren’s syndrome
- Rheumatoid arthritis
- Raynad’s phenomena: During which your ears and fingers become frostbitten and changes color
- Cardiovascular problems:
- Inflammation of the heart muscle or myocarditis
- Heart failure
- Arrhythmias
- Respiratory lung problems:
- Interstitial pulmonary disease
- Lung fibrosis
- Cancers:
It is said that a person, especially an elderly person, is more likely to get cancer three years after the onset of dermatomyositis, so in people with dermatomyositis, possible tests should be done to diagnose the cancer. And it is interesting that in some people this cancer exists and causes dermatomyositis.