What is Dermatomyositis?
Myopathies are a group of diseases that involve chronic muscle inflammation and weakness. Some people think they might be autoimmune diseases - which means the body's natural defenses (antibodies, lymphocytes, etc.) start attacking healthy tissue without reason. Inflammation or swelling can occur for reasons that are unknown.
Explanation of medical terms and
concepts Dermatomyositis
Dermatomyositis is an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash
Dermatomyositis is a condition that affects adults and children In adults this condition usually occurs in the late 40s to early 60s In children it most often appears between 5 and 15 years of age Dermatomyositis affects more females than males
There is no cure for dermatomyositis but there can be improvement of symptoms Treatment can help clear the skin rash and help regain muscle strength and function
Symptoms Dermatomyositis
The symptoms of dermatomyositis can be gradual or sudden. Sometimes the symptoms will wax and wane without any apparent reason.
The main symptom of the disorder is muscle weakness that often affects the trunk and muscles closest to the trunk (for example, proximal muscles), such as the hips, thighs, shoulders, upper arms, and neck. The affected muscles may be stiff, sore, and/or tender, and eventually they may show signs of degeneration. Muscle wasting (atrophy) can cause weakness and degeneration of muscles, which can eventually lead to an awkward gait and difficulty performing certain tasks such as lifting your arms or climbing steps. Some common signs may include an inability to raise your head from the ground, or a gradual decline in muscle strength. A pillow will help you to lie down or rise from the floor without assistance. In some people with this disorder, involvement of muscles in the neck, tongue, and/or throat may eventually lead to difficulties swallowing (dysphagia) and/or articulating speech (dysphonia). In rare cases, weakening of muscles in the chest can occur. Respiratory difficulties can occur if the wall and diaphragm are not properly treated. These difficulties can lead to life-threatening complications without prompt treatment. In addition, in some people with chronic long-term illness, certain joints may become permanently bent (flexed) and cause contractures. If a second grader has problems walking, it might be because he or she is not getting enough exercise.
Some people with dermatomyositis also develop distinctive skin changes that may be the only sign of the disease at the start in up to 40% of people. Skin abnormalities often include a distinctive reddish-purple or blue appearance. A lilac rash may be present on the upper eyelids (called heliotrope eyelids), the forehead, the cheeks and bridge of the nose (called “butterfly rash”), and/or other regions. In addition, affected skin may be present on the extensor surfaces of certain joints, such as the knuckles, elbows, knees, or hips. Other regions may have scaly skin with central areas of tissue loss (atrophy) and a decreased pigmentation (Gottron sign). A "dusky" reddish (erythematous) rash may also develop on the upper arms, legs, and trunk. The rash can be itchy and lead to People may have trouble sleeping because of skin rashes. Although these rashes often disappear completely, they may occasionally result in brownish discolorations (hyperpigmentation) on the skin, atrophy of scars, or loss of color (depigmentation) in patches of skin. Dermatomyositis may also be associated with this problem. Some changes that may occur to the nails include blue-red scaling lesions around the base of the nails; reddish "shininess" of the nail folds; widening (dilation) of capillaries in the nail bed; or other findings. Other skin manifestations may include abnormal growths or changes. Chronic disease can cause accumulation of fluid in body tissues (edema), around the eyes (periorbital area) and other facial regions. This can lead to increased sensitivity to light (photosensitivity), and/or other findings. In some cases, long-term chronic disease may result in weakening of the skin, which can bind to underlying tissues. Some people may be affected by this condition, which may lead to abnormal accumulations of calcium in muscles and tissues under the skin. This can cause contractures and localized muscle weakness. People may also experience itching and scaling lesions. Head lice can develop and result in significant hair loss.
Some people with dermatomyositis may also develop symptoms such as a low-grade fever, feeling of ill health (malaise), shortness of breath (dyspnea), pain in multiple joints (polyarthralgia), weight loss, and/or other abnormalities. In some cases, affected individuals may particularly those who have it more severely, for example those who also have low blood pressure or problems with their kidneys. People with muscle inflammation (myositis) and other connective tissue disorders often experience Raynaud phenomenon. This is a condition in which sudden contractions of blood vessels supplying the fingers and toes (digits) causes a temporary interruption of blood flow, causing numbness. If you have tingling or painful episodes that occur most commonly when you are exposed to cold temperatures, you may have Raynaud's disease.
Muscle weakness and impairment can progress and affect other areas of the body, including muscles in the digestive system (gastrointestinal blood vessels and circulation), as well as the lungs. In rare cases, the heart muscle may weaken (cardiomyopathy) or experience irregularities. Patients with dermatomyositis can experience arrhythmias (abnormal heart rhythms), inflammation of the lung tissue, and breathing difficulties (dyspnea) as well as plaque accumulation in the arteries.
Some people with dermatomyositis may have an underlying cancer. Reports suggest that the cancer may occur before or after the onset of dermatomyositis. Malignancy-associated dermatomyositis appears to happen in some people. Cancer tends to occur more frequently in individuals over the age of 40-50. However, experts do not know which specific areas of the body cancer typically begins. Some common sites that are suspected include the GI tract (esophagus, stomach, small intestine), lungs, breastbone, and testicles. We don't fully understand the relationship between dermatomyositis and cancer. Some cancers may be related to dermatomyositis, but we don't know for sure.
The symptoms and physical findings associated with childhood dermatomyositis are similar to those observed in adult dermatomyositis. Childhood dermatomyositis typically occurs more suddenly than in the adult form, and often involves skin manifestations followed by muscle weakness. Muscle calcification may occur. Dermatomyositis is more common in children than in adults. The deposits often look like white or flesh-colored nodules over bone areas, and they usually develop within three years. A diagnosis of celiac disease is only a starting point, but it can develop up to 20 years later. Children with celiac disease typically have widespread inflammation of blood vessels (vasculitis) as well as GI symptoms such as abdominal pain, difficulty swallowing, and frequent vomiting or diarrhea. When something goes wrong with the digestive system, children may experience constipation, black stools (melena), or bleeding from the stomach (peptic ulcers). Children may also develop a limp because of stiffness in the ankles. If something is wrong with the child's body, it is called a malignancy. Dermatomyositis is not commonly associated with childhood.
Sometimes people with dermatomyositis have skin abnormalities, without the muscle problems.
Most symptoms of dermatomyositis appear suddenly or develop gradually over time The most common signs and symptoms include:
- Skin changes.A violet-colored or dusky red rash develops most commonly on your face eyelids and knuckles elbows knees chest and back The rash which can be itchy and painful is often the first sign of dermatomyositis
- Muscle weakness.Progressive muscle weakness involves the muscles closest to your trunk It affects both sides of your body and gradually worsens
When to see a doctor
If you develop muscle weakness or an unexplained rash seek medical attention
Causes Dermatomyositis
We don't know the specific underlying cause of dermatomyositis, but evidence suggests that genetic and environmental factors play a role. Underlying genetic and immune mechanisms may be suggested by findings such as an increased frequency of certain genetically determined conditions. HLAs are proteins that play an important role in the body’s immune system. People with the disorder have higher levels of HLA proteins. This may influence whether or not a person is predisposed to certain diseases. There is evidence that suggests Some HLAs are more common in children and adults with dermatomyositis. However, we don't know yet what the specific implications of these findings are.
Dermatomyositis is thought to be an autoimmune disorder in which the body's natural immune defenses attack the body's own tissues. This abnormal reaction appears to lead to obstructive inflammatory changes in blood. Muscles have small vessels within them that connect different parts of the skin and other tissues. As this tissue degenerates, it can result in wasting (atrophy) and new muscle growth. The outermost layer of the skin may also thin, and other associated findings may occur.
Some researchers believe that certain infectious agents (such as coxsackie virus, parvovirus, echovirus, and HIV) may be potential triggers for dermatomyositis. Some people believe that people with a genetic predisposition are more likely to develop this condition. If someone has a virus, they may have proteins in their blood that are similar to some of the body's own proteins. This could cause the body to make antibodies. These antibodies can incorrectly attack the body's own cells (intramuscular blood vessels), which can lead to an infection. Inflammatory changes and muscle tissue damage are often seen in dermatomyositis. Environmental factors and weather have also been implicated in cases of juvenile dermatomyositis that develop or become worse in the months of April and May. In addition, researchers believe that the association of dermatomyositis with It's possible that underlying cancers in some adults may be the result of an abnormal autoimmune response against a common antigen, such as a tumor in muscle. There have also been reports of dermatomyositis occurring after certain vaccinations or the use of medications. The implications of these findings remain unknown, so more data is needed to determine if there is an association between silicone breast implants and collagen injections and dermatomyositis
The cause of dermatomyositis is unknown but it has much in common with certain autoimmune disorders in which your immune system mistakenly attacks the tissues of your body
Environmental factors may include viral infections sun exposure certain medications and smoking
Complications
Potential complications (side effects) of Dermatomyositis include:
- Difficulty swallowing.
- Aspiration pneumonia.Difficulty swallowing can also cause you to breathe food or liquids into your lungs
- Breathing problems.If the condition affects your chest muscles you might develop breathing problems such as shortness of breath
- Calcium deposits.These deposits can occur in your muscles and skin as the disease progresses They are more common in children with dermatomyositis and develop earlier in the course of the disease
Associated conditions
Dermatomyositis might cause other conditions or put you at higher risk of developing them including:
- Raynaud's phenomenon.This condition causes fingers toes cheeks nose and ears to turn white in cold temperatures
- Other connective tissue diseases.Other conditions such as lupus rheumatoid arthritis scleroderma and Sjogren's syndrome can occur with dermatomyositis
- Cardiovascular disease.Dermatomyositis can cause heart muscle inflammation in a small number of people who have it In these cases some people may develop congestive heart failure and arrhythmias (irregular heart rhythms)
- Lung disease.Interstitial lung disease refers to a group of disorders that cause scarring of the lungs making the lungs stiff and inelastic Signs include a dry cough and shortness of breath
- Cancer.Some people with dermatomyositis develop cancer particularly ovarian cancer in women Risk of cancer appears to level off three years or so after a diagnosis of dermatomyositis
Dermatomyositis has similar words that mean the same thing.
- ADM
- childhood dermatomyositis
- idiopathic inflammatory myopathy
- IIM
- polymyositis
Subdivisions of Dermatomyositis
- adult dermatomyositis
- dermatomyositis sine myositis
- JDMS is a disease that affects children's skin.
General Discussion
Diagnosis Dermatomyositis
To diagnose dermatomyositis, a detailed patient history and careful clinical examination are needed. The presence of a characteristic skin rash and progressive muscle weakness can be detected. High levels of certain muscle enzymes (such as creatine kinase and aldolase) in the liquid portion of the blood (serum); and abnormalities on electromyography (a test that records muscle activity). These findings may be indicative of muscle inflammation. Doctors can use various tests to see how muscles are working, such as measuring electrical activity in the muscles at rest and during muscle contraction. They may also recommend MRI scans to see details inside the tissues. Sometimes biopsies may be recommended to help confirm a diagnosis. Muscle biopsy involves the removal and microscopic examination of small samples of muscle tissue. Experts say that muscles recently tested with EMG should not be used as samples, because EMG procedures can damage the muscle. A biopsy may show signs of inflammation, which could lead to a false positive result. Additional imaging studies may be performed in order to confirm the diagnosis, including: CT scans of the chest, radiographs of the lungs, a barium swallow, and an ultrasound of the muscle involved.
Some blood tests may provide helpful information that suggests a diagnosis, but the results may not be specific - meaning they may be associated with a variety of autoimmune disorders. For example, some people with this condition may have an elevated level of something. ESR measures the rate at which red blood cells (erythrocytes) settle to the bottom of a test tube. Because erythrocytes tend to cluster together and thus settle more quickly when inflammation is present, an elevated ESR is an nonspecific indicator of inflammation. Inflammation. In some people, testing may also show certain antinuclear antibodies (ANAs) which are "self-antibodies" (autoantibodies) that react with antigens in the nucleus of cells. A number of specific autoantibodies or myositis-specific antibodies have been identified. Some people with inflammatory muscle diseases, such as dermatomyositis and polymyositis, may have anti-Jo-1 antibodies. This can help classify the disease in certain cases.
Sometimes additional tests may be recommended to help identify or characterise certain abnormalities associated with the disorder. For example, x-rays may reveal calcifications in soft tissues. Stool samples may be collected to detect blood, which could indicate a medical condition. The symptoms described might suggest a gastrointestinal or pulmonary problem. A lung function test may also be performed to assess for involvement in the lungs.
When examining adults for dermatomyositis, the possibility of an underlying malignancy should be considered. This includes a basic physical examination, including breast gynecologic and/or rectal examination screening. Blood testing may be done to look for abnormalities in urine. Women may have x-rays of their breasts, as well as other tests, in order to determine if they have cancer. They will also need to have follow-up testing to make sure the cancer has not spread.
To diagnose juvenile dermatomyositis, you must meet the following criteria: a characteristic skin rash; generalized muscle weakness; high muscle enzymes; and/or abnormal muscle biopsy findings.
If you have dermatomyositis your doctor might suggest some of the following tests:
- Blood analysis.A blood test could reveal whether you have high levels of muscle enzymes that can indicate muscle damage A blood test can also detect autoantibodies associated with different symptoms of dermatomyositis and can help your doctor determine the best medication and treatment
- Chest X-ray.This simple test can detect the type of lung damage that occurs in some patients who have dermatomyositis
- Electromyography. A doctor inserts a thin needle through the skin into the muscles to be tested Electrical activity is measured as you relax or tighten the muscle and changes in the pattern of electrical activity can confirm a disease The doctor can determine which muscles are affected by that disease are affected by it
- MRI.An MRI scanner creates cross-sectional images of muscles The data involved in an MRI scans are generated by a powerful magnetic field and radio waves An MRI scan can assess inflammation over a large area of muscle unlike a muscle biopsy which can only detect the presence or absence of inflammation and is unable to detect the degree of inflammation
- Skin or muscle biopsy. A small piece of skin or muscle is removed for laboratory analysis Skin biopsy can help confirm the diagnosis of dermatomyositis Muscle biopsy might reveal inflammation in your muscles or other problems such as damage or infection If the skin biopsy confirms the diagnosis a muscle biopsy will be taken from that area to confirm Some people might not be necessary
Treatment Dermatomyositis
There's no cure but treatment can improve your skin and muscle strength and function
Medications
The medications used to treat dermatomyositis include:
- Corticosteroids.Prednisone (pred-niz-oh) can control dermatomyositis symptoms quickly but it has serious side effects Your doctor might start you on a high dose to control your symptoms and then gradually reduce the dosage as your disease improves
- Corticosteroid-sparing agents. If used with a corticosteroid these drugs can significantly decrease the dose of the corticosteroid and possibly alleviate side effects The two most common medications for dermatomyositis are azathioprine (Azasan Imuran) and methotrexate (Trexall) Mycophenolate mofetil (Cellcept) is another medication used to treat dermatomyositis a disease of connective tissue which is characterized by inflammation and damage to the skin lung and bone marrow
- Rituximab (Rituxan).Rituximab is an option if initial therapies don't control your symptoms
- Antimalarial medications.For a persistent rash your doctor might prescribe an antimalarial medication such as hydroxychloroquine (Plaquenil)
- Sunscreens.
Therapy
If you have severe symptoms your doctor might suggest:
- Physical therapy.A physical therapist can help you maintain and improve strength and flexibility He or she may also advise you about an appropriate level of activity
- Speech therapy. If your swallowing muscles are affected, speech therapy can help you learn how to compensate for those changes.
- Dietetic assessment.Later in the course of dermatomyositis swallowing may become more difficult A registered dietitian can teach you how to prepare easy-to-eat foods
Surgical and other procedures
- Intravenous immunoglobulin (IVIg). IVIg is a purified blood product that gives healthy antibodies from thousands of blood donors These antibodies can block the damaging antibodies that attack muscle and skin in dermatomyositis Given as an infusion through a vein IVIg treatments are expensive and might need to be repeated to continue taking the medication regularly
- Surgery.Surgery might be a way to take out painful calcium deposits and prevent recurrent skin infections
Standard Therapies
Lifestyle and home remedies
With dermatomyositis affected areas are more sensitive to the sun Wear a hat or use sunscreen when you go outside
Coping and support
Living with a chronic autoimmune disease can make you wonder whether you are up to the challenge To help you cope try the following:
- Know your illness.Find out more about dermatomyositis and other muscle and autoimmune disorders Speak with people who have a similar condition Don't be afraid to ask your doctor questions concerning your diagnosis or treatment plan
- Be a part of your medical team.You should keep your doctor updated on any new signs or symptoms you develop Those changes might indicate that your treatment plan needs to change
- Get active.Regular exercise can help you maintain and build your muscle strength Be sure to get a detailed plan and recommendations from your doctor or physical therapist before starting an exercise program
- Rest when you're tired.You should not wait until you are exhausted to learn a new skill or develop a new habit as this will only set you back further Instead learn to pace yourself so that you can maintain your energy level and accomplish just as much as before and feel better emotionally
- Acknowledge your emotions.Denial anger and frustration are normal when you have dermatomyositis You may feel fear and isolation as well Try to maintain your daily routine especially keeping up with the things that bring you joy Many people find support groups helpful in dealing with this illness
Preparing for your appointment
You probably first see a general practitioner who might refer you to a doctor who specializes in treating arthritis and other diseases of the joints and muscles (rheumatologist) and to a doctor who specializes in skin diseases (dermatologist)
What you can do
Make a list that includes:
- Detailed descriptions of your symptoms and when they began
- Information about medical problemsyour parents and siblings have had things that you do not know about
- All of these medicines and dietary supplements are prescribed by doctors in the same way you take and their doses
- Questions to ask the doctor
Take a friend or family member along if possible to help you remember the information you are given
Skin diseases questions to ask your doctor include:
- What is likely causing my symptoms?
- What are other possible causes?
- Is my condition likely to change over time?
- What tests might I need? What preparations should I make?
- There are treatments for my condition What treatments would you recommend?
- I have other medical conditions How can I best manage them together?
- Do you have any promotional material or printed materials I can have? What websites do you recommend?
Ask questions of the instructor if you don't understand something
What to expect from your doctor
Your doctor will likely ask you questions such as:
- Has your condition worsened or gotten worse gradually or suddenly?
- Are you easily fatigued during waking hours?
- Is your condition limiting your activities?
- Has anyone in your family been diagnosed with a condition that affects the muscles? (this is a more formal version of “Have you ever had muscle pain/weakness?)
- What if you have any improvement?
- What if anything appears to make your symptoms worse?
Affected Populations
Dermatomyositis can occur at any time, but it is most common in people between the ages of 40 and 60. The estimated incidence of dermatomyositis is 9.63 cases per million people. In children, the symptoms usually appear between the ages of five and 15 years. Juvenile dermatomyositis affects three out of every hundred thousand children. Females are affected twice as often as males.
General summary
Dermatomyositis is a rare disorder that causes rashes and muscle weakness Dermatomyositis primarily affects children and young adults typically between the ages of 10 and 30 years old The disease can be categorized into 3 different stages: mild (also known as juvenile dermatomyositis) intermediate and severe Fortunately most patients recover from the first or mild stage within six months to two years However those with severe stage may experience severe disability or even death resulting rarely from this condition Sadly treatments only work in some cases and there is no cure associated with it yet
Symptoms Dermatomyositis is a rare disease that affects the skin and muscles This condition can cause tell-tale signs such as skin rashes muscle weakness excessive fatigue and joint pain The symptoms vary according to the severity of the condition with mild cases causing few or no symptoms Dermatomyositis most often affects infants and young children though it can also occur in adulthood
Can dermatomyositis be cured?
Dermatomyositis cannot be cured completely but symptoms can be managed For most patients the disease will eventually progress and worsen over time Current medical treatments are aimed at reducing signs and symptoms of this disease
An immune system problem called dermatomyositis is the main cause of a skin rash in 90 percent of cases In some people this disease damages muscles as well as the skin Symptoms include fever weakness and fatigue as well as muscle aches joint pains stiffness and loss of appetite About five to seven percent of cases are caused by a connective tissue disorder known as polymyositis that affects only the muscles in the body rather than both skin and muscles in dermatomyositis The remaining 10 to 15 percent are caused by an autoimmune condition known as anti-phospholipid syndrome (APS) that causes
What is the life expectancy of dermatomyositis?
People with dermatomyositis have an average life expectancy of about 30 years but 10% will progress to a related disease amyotrophic lateral sclerosis (ALS) that usually results in death within five years
How serious is dermatomyositis?
Dermatomyositis is an uncommon but serious autoimmune connective tissue disease that causes muscle weakness It can affect muscles throughout the body including those in the face and extremities (arms and legs) The weakening of these muscles leads to problems with mobility and a loss of strength needed for daily activities Excerpt from:
Can you live a normal life with dermatomyositis?
Dermatomyositis is a rare autoimmune disease that affects the muscles and sometimes causes skin problems There is no specific cure for this condition so treatment focuses on managing the symptoms of dermatomyositis While living with dermatomyositis can be difficult it is possible to live a normal life Treatment may help ease symptoms such as pain and fatigue but patients should learn to manage their expectations because they will probably not improve completely
What organs does dermatomyositis affect?
The main organs affected by dermatomyositis are the skin muscle and joints Dermatomyositis causes inflammation that affects the skin in many ways The most obvious symptom is a rash which can appear anywhere on the body but typically appears on the face neck trunk and shoulders Swollen lymph nodes especially around the eyes are also common
For patients with dermatomyositis treatment options are limited There is no cure for this condition and many people who develop it will experience relapses For the most part treatments involve using medications to deal with symptoms of muscle weakness and fatigue associated with this condition This may include steroids to help alleviate pain caused by inflammation as well as side effects of some medications that can occur or surgery for those who have developed heart damage from this disease