What is the pathophysiology of dermatomyositis?
Dermatomyositis is one of a group of rare muscle diseases called inflammatory myopathies, which are characterized by chronic muscle inflammation accompanied by muscle weakness. An identifying factor for dermatomyositis is a skin rash that precedes or accompanies progressive muscle weakness.
Which muscles are most affected by dermatomyositis?
The muscles of the shoulders, upper arms, hips, thighs and neck display the most weakness in dermatomyositis. There also can be joint pain, inflammation of the heart and lung muscle tissues, as well as inflammation of blood vessels to other organs.
What are the possible complications of dermatomyositis?
Possible complications for some people with dermatomyositis include lung disease, heart disease, or cancer. These can make treatment more difficult. If you have dermatomyositis, you may need to be treated for the rest of your life. It's important to learn as much as you can about the disease. Work closely with your health care provider.
Is dermatomyositis a rare disease?
Dermatomyositis is a rare disease. A rare disease in the U.S. is defined as a condition affecting fewer than 200,000 people. Here is some additional information: DM affects twice as many women as men. DM is more common in African Americans, who, according to some studies, are affected at three-times the rate of the Caucasian population in the U.S.
How long can you live with dermatomyositis?
For dermatomyositis, polymyositis, and necrotizing myopathy, the progression of the disease is more complicated and harder to predict. More than 95 percent of those with DM, PM, and NM are still alive more than five years after diagnosis.
What organs does dermatomyositis affect?
The muscles of the shoulders, upper arms, hips, thighs and neck display the most weakness in dermatomyositis. There also can be joint pain, inflammation of the heart and lung muscle tissues, as well as inflammation of blood vessels to other organs. For more, see Signs and Symptoms.
Do neurologists treat dermatomyositis?
Patients with dermatomyositis, polymyositis, or necrotizing myopathy are usually treated by rheumatologists. Those with dermatomyositis may also work with a dermatologist. Those with IBM are often treated by neurologists.
What happens if dermatomyositis is not treated?
Dermatomyositis in adults has been linked to an increased likelihood of developing cancer, particularly ovarian cancer in women. Risk of cancer appears to level off three years or so after a diagnosis of dermatomyositis.
What triggers dermatomyositis?
Reports indicate that certain infectious agents (i.e. coxsackie virus, parvovirus, echovirus, HIV, human T-cell lymphotrophic virus Type 1, and Toxoplasma and Borrelia species) have been suggested as potential triggers for dermatomyositis.
What is the death rate of dermatomyositis?
Previous studies reported that the connective tissue disease PM/DM has a poor prognosis and high I-HMR. The reported 10-year survival rate ranged between 53% and 91% [4, 9–12]. A very recent population-based study from America reported a hospital mortality of 4.5% [2].
Is there a blood test for dermatomyositis?
Dermatomyositis is usually diagnosed with blood tests and biopsies of your skin and muscles. Your provider will test your blood for: Increased amounts of specific muscle enzymes that means something is damaging them. Autoantibodies (cells that show your immune system is reacting to something it detects as harmful).
Is dermatomyositis a progressive disease?
Childhood dermatomyositis describes a group of muscle diseases characterized by chronic muscle inflammation followed by progressive muscle weakness.
How does dermatomyositis affect the eyes?
1.1 Disease Heliotrope eyelid eruptions are considered a hallmark of DM affecting the eye. Other ocular manifestations of DM include conjunctival edema, nystagmus, extraocular muscle weakness, iritis, cotton wool spots, optic atrophy, and conjunctival pseudopolyposis. Retinopathy is also a rare presentation.
Can Covid trigger dermatomyositis?
COVID-19 can induce a systemic inflammatory response, and its clinical manifestations are diverse. Recently, it has been reported that COVID-19 patients may develop myositis and interstitial pulmonary disease similar to dermatomyositis (DM).
Does dermatomyositis qualify for disability?
Dermatomyositis has been identified by the Social Security Administration as one of the conditions that can qualify a person to receive Social Security Disability Insurance or Supplemental Security Income.
Does exercise help dermatomyositis?
Exercise can improve mitochondrial function, angiogenesis as well as improve muscle growth and reduce inflammation in established polymyositis and dermatomyositis.
What does a dermatomyositis flare up feel like?
Dermatomyositis is a rare disease that causes muscle weakness and skin rash. Symptoms include a red or purple rash on sun exposed skin and eyelids, calcium deposits under the skin, muscle weakness, and trouble talking or swallowing. There is no cure, but treatment is done to reduce the symptoms.
Does dermatomyositis affect your eyes?
Heliotrope eyelid eruptions are considered a hallmark of DM affecting the eye. Other ocular manifestations of DM include conjunctival edema, nystagmus, extraocular muscle weakness, iritis, cotton wool spots, optic atrophy, and conjunctival pseudopolyposis. Retinopathy is also a rare presentation.
Can dermatomyositis cause death?
Dermatomyositis is a serious illness that in rare cases can be fatal. It's important to get diagnosed and start treatment as early as possible. While there is no cure for dermatomyositis, symptoms can often be managed with long-term (sometimes life-long) medications and physical therapy.
Is dermatomyositis related to lupus?
The association of systemic lupus erythematosus (SLE) with idiopathic polymyositis or dermatomyositis is reported to occur in the range of 4-16%. Myositis can occur before or after SLE, or sporadically both diseases can be present simultaneously.
What are the symptoms of dermatomyositis?
The symptoms are caused by swelling and inflammation in the blood vessels that supply your skin and muscles, and can include:
How to treat dermatomyositis?
Treatment. There is no cure for dermatomyositis, but you can treat the symptoms with medication, physical therapy, exercise, heat therapy and rest. Medications include corticosteroids, immunosuppressant drugs and topical ointments.
How is dermatomyositis diagnosed?
The process starts with a person’s medical history and a physical exam. The health care provider will look for an underlying disease, such as cancer. Tests may also be done, such as:
What is the condition that causes rash and inflammation?
What is dermatomyositis? Dermatomyositis is a rare disease that causes muscle inflammation and skin rash. It’s one of a group of muscle diseases that cause muscle inflammation and swelling. It's different from other muscle diseases because it also causes skin problems.
Why does my immune system attack my own tissues?
The exact cause is not known, but possible causes include: Autoimmune disease, a type of illness that causes the body’s immune system to attack its own tissues. An infection, medication, or another exposure in your environment that triggers the disease.
What are the symptoms of muscle weakness?
Muscle weakness in the neck, hip, back, and shoulders. Trouble swallowing and voice changes. Tiredness, fever, and weight loss. Muscle aches. Trouble rising from a chair or getting out of bed due to muscle weakness. Sometimes the muscle weakness also spreads to the heart, GI tract, and lungs.
What are the symptoms of dermatomyositis?
The most common signs and symptoms include: Skin changes. A violet-colored or dusky red rash develops, most commonly on your face and eyelids and on your knuckles, elbows, knees, chest and back. The rash, which can be itchy and painful, is often the first sign of dermatomyositis. Muscle weakness.
How old is the best age to get dermatomyositis?
In children, it most often appears between 5 and 15 years of age. Dermatomyositis affects more females than males. There's no cure for dermatomyositis, but periods of symptom improvement can occur. Treatment can help clear the skin rash and help you regain muscle strength and function.
What is the name of the disease that causes muscle weakness and rash?
Dermatomyositis (dur-muh-toe-my-uh-SY-tis) is an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash.
What is progressive muscle weakness?
Muscle weakness. Progressive muscle weakness involves the muscles closest to the trunk, such as those in your hips, thighs, shoulders, upper arms and neck. The weakness affects both the left and right sides of your body, and tends to gradually worsen.
Can dermatomyositis cause heart problems?
Cardiovascular disease. Dermatomyositis can cause heart muscle inflammation. In a small number of people who have dermatomyositis, congestive heart failure and heart rhythm problems develop.
Does dermatomyositis cause cancer?
Cancer. Dermatomyositis in adults has been linked to an increased likelihood of developing cancer, particularly ovarian cancer in women. Risk of cancer appears to level off three years or so after a diagnosis of dermatomyositis. By Mayo Clinic Staff.
How to treat dermatomyositis?
Treatment. There is no cure for dermatomyositis, but the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. The standard treatment for dermatomyositis is a corticosteroid drug, given either in pill form or intravenously.
What is the inflammatory myopathy?
Dermatomyositis is one of a group of muscle diseases known as the inflammatory myopathies, which are characterized by chronic muscle inflammation accompanied by muscle weakness. Dermatomyositis’ cardinal symptom is a skin rash that precedes, accompanies, or follows progressive muscle weakness. The rash looks patchy, with purple or red discolorations, and characteristically develops on the eyelids and on muscles used to extend or straighten joints, including knuckles, elbows, knees, and toes. Red rashes may also occur on the face, neck, shoulders, upper chest, back, and other locations, and there may be swelling in the affected areas. The rash sometimes occurs without obvious muscle involvement. Adults with dermatomyositis may experience weight loss, a low-grade fever, inflamed lungs, and be sensitive to light such that the rash or muscle disease gets worse. Children and adults with dermatomyositis may develop calcium deposits, which appear as hard bumps under the skin or in the muscle (called calcinosis). Calcinosis most often occurs 1-3 years after the disease begins. These deposits are seen more often in children with dermatomyositis than in adults. In some cases of dermatomyositis, distal muscles (muscles located away from the trunk of the body, such as those in the forearms and around the ankles and wrists) may be affected as the disease progresses. Dermatomyositis may be associated with collagen-vascular or autoimmune diseases, such as lupus.
Is dermatomyositis resistant to therapy?
Most cases of dermatomyositis respond to therapy. The disease is usually more severe and resistant to therapy in individuals with cardiac or pulmonary problems.
Can dermatomyositis cause weight loss?
Adults with dermatomyositis may experience weight loss, a low-grade fever, inflamed lungs, and be sensitive to light such that the rash or muscle disease gets worse. Children and adults with dermatomyositis may develop calcium deposits, which appear as hard bumps under the skin or in the muscle (called calcinosis).
Other muscle groups
The condition may also affect other muscle groups, such as the throat, tongue, chest, and heart. This can lead to:
Skin changes
In 40% of people, skin changes may precede muscle weakness. The two hallmark features of the condition are Gottron’s papules and a heliotrope rash.
Additional symptoms
calcinosis, which is the abnormal accumulation of calcium deposits in muscle and skin tissues
Genetic factors
People with particular human leukocyte antigen (HLA) types are at a higher risk of the condition.
Environmental factors
Doctors observe this condition more frequently in females with exposure to high frequency UV radiation.
Lung disease
One-third of people with dermatomyositis have interstitial lung disease, which involves inflammation and scar tissue in the lungs. A person with respiratory muscle weakness may also develop aspiration pneumonia.
Connective tissue diseases
Dermatomyositis can occur together with other connective tissue diseases, such as:
How to diagnose dermatomyositis?
The diagnosis of dermatomyositis is usually confirmed by the following tests: 1 Blood tests to detect increased amounts of muscle enzymes such as creatine kinase (CK) and sometimes lactic dehydrogenase (LDH). 2 Blood tests to detect autoantibodies (antibodies that react with cells, tissues, or native proteins of the individual in which the antibodies are produced). 3 Skin biopsy of the rash. 4 Biopsy of an affected muscle. 5 Electromyography (EMG) testing. 6 Magnetic resonance imaging (MRI) scan of muscles.
What test is used to diagnose dermatomyositis?
The diagnosis of dermatomyositis is usually confirmed by the following tests: Blood tests to detect increased amounts of muscle enzymes such as creatine kinase (CK) and sometimes lactic dehydrogenase (LDH).
What is the term for a condition in which patients have the characteristic cutaneous findings of dermatomyos?
Clinic ally amyopathic dermatomyositis(CADM) is a condition in which patients have the characteristic cutaneous findings of dermatomyositis, but do not have muscle weakness. Clinically amyopathic dermatomyositis is further classified as hypomyopathicor amyopathicdermatomyositis.
What is a rare acquired immune-mediated muscle disease characterized by muscle weakness and skin rash?
Dermatomyositis is a rare acquired immune-mediated muscle disease characterized by muscle weakness and skin rash. It is classified as one of the idiopathic inflammatory myopathies (IIM).
Does dermatomyositis affect other organ systems?
Also, it can affect other organ systems such as the pulmonary, cardiovascular, and gastrointestinal systems. A significant proportion of patients with dermatomyositis have an underlying malignancy, which can alter the prognosis of the condition.
Is Dermatomyositis an immune disease?
Dermatomyositis is a rare acquired immune-mediated muscle disease characterized by muscle weakness and skin rash. It is classified as one of the idiopathic inflammatory myopathies (IIM). Although all idiopathic inflammatory myopathies share the common presentation of muscle weakness, they differ clinically in terms of muscle groups involved ...
Can hypomyopathic dermatomyositis be a muscle weakness?
Patients with hypomyopathic der matomyositis lack muscle weakness clinically. However, there is evidence of myositis based on laboratory investigations, electromyography, or muscle biopsy. In contrast, patients with amyopathic dermatomyositis lack both clinical and laboratory evidence of muscle involvement.
What is myopathy and myositis?
Understanding myopathy and myositis. Myopathy and myositis are neuromuscular conditions that cause muscle problems, such as stiffness or weakness. Many people with these conditions have not been diagnosed or may have been misdiagnosed with another illness.
Which type of myositis affects more women than men?
Gender: Two types, dermatomyositis and polymy ositis, affect more women than men. A different type, inclusion body myositis, affects more men, usually over age 50.
What is the ALS and Neuromuscular Disease Center?
The ALS and Neuromuscular Disease Center provides complete care for nerve and muscle conditions, including all types of myopathy and myositis.
What is a muscle disease that causes muscle stiffness?
Myopathies: These are muscle diseases in which the muscle fiber doesn’t work properly. Myopathies cause problems with the muscles you use for voluntary movements such as walking or trying to grab something. Patients often have muscle stiffness or weakness.
What is the immune system attack?
Polymyositis: The immune system attacks muscle fibers. Muscle weakness makes it difficult to rise from a seated position or lift objects. Inclusion body myositis: In addition to inflammation, people with inclusion body myositis also experience loss of muscle mass. This disease generally affects adults ages 50 and older.
What are the three rare conditions that cause a rash under the skin?
Types of myositis. These three rare conditions are also known as inflammatory myopathies because the muscles become inflamed and weak. Dermatomyositis: The immune system attacks blood vessels that supply the muscles and skin, causing a skin rash along with hard bumps under the skin.
What is inherited myopathies?
Other types, called inherited myopathies, are caused by a genetic change passed down from parents. OHSU offers expert genetic testing and counseling to help you and your family identify and manage risk. Autoimmune diseases, when the body’s immune system mistakenly attacks its own muscles.
What causes dermatomyositis?
In dermatomyositis, these cells attack the small blood vessels that supply muscles and skin.
What are the factors that trigger dermatomyositis?
Scientists are examining factors that may trigger dermatomyositis such as viruses, certain drugs or vaccines.
What is the root of myositis?
Another word for inflammatory myopathy is myositis. The myo root means muscle, and the itis root means inflammation; so a myositis is an inflammatory muscle disease.
What is the name of the disease that involves the blood vessels that supply the muscles?
Dermatomyositis is one of the inflammatory myopathies, a group of muscle diseases that involves inflammation of the muscles or associated tissues, such as the blood vessels that supply the muscles. A myopathy is a muscle disease, and inflammation is response to cell damage.
What is the condition where calcium is deposited under the skin?
Other symptoms of dermatomyositis include a condition called calcinosis, in which calcium is deposited just under the skin in hard, painful nodules, and panniculitis, inflammation of the fat lying just under the skin.
When does dermatomyositis start?
Onset of dermatomyositis occurs in childhood or adulthood, and its progression is gradual. In children, onset is usually between the ages of 5 and 14, and girls are affected more often than boys.
Which muscle has the most weakness?
The muscles of the shoulders, upper arms, hips, thighs and neck display the most weakness in dermatomyositis. There also can be joint pain, inflammation of the heart and lung muscle tissues, as well as inflammation of blood vessels to other organs. For more, see Signs and Symptoms.
Who is affected by Dermatomyositis?
Dermatomyositis is a rare disease. A rare disease in the U.S. is defined as a condition affecting fewer than 200,000 people.
What are the symptoms of dermatomyositis?
Not all patients display all of these symptoms. Skin rashes and discolorations which affect sun-exposed areas, cheeks, nose, shoulders, upper chest, and elbows. Proximal muscle weakness (muscles closest to the trunk, such as shoulders, thighs, and hips)
What is the identifying factor for dermatomyositis?
An identifying factor for dermatomyositis is a skin rash that precedes or accompanies progressive muscle weakness. Muscle weakness, when present, can develop over a period of days, weeks, or months.
What is DM in medical terms?
Dermatomyositis (DM) Dermatomyositis is one of a group of rare muscle diseases called inflammatory myopathies, which are characterized by chronic muscle inflammation accompanied by muscle weakness. An identifying factor for dermatomyositis is a skin rash that precedes or accompanies progressive muscle weakness.
Is dermatomyositis a rash?
A skin rash that precedes or accompanies muscle weakness is often helpful in diagnosing DM. Muscle involvement, for some, is not a part of their disease. Muscle pain (myalgia) and joint pain, with or without true arthritis and joint inflammation, can also be a part of dermatomyositis. DM is a chronic, incurable disease.
Is a rash painful?
Some rashes may be painful, severely itchy (pruritis), and intense enough to disturb sleep. Scalp inflammation and thinning of the hair is also common. In rare cases, cutaneous vasculitis, ulcerations, and calcinosis are observed, although more so with juvenile dermatomyositis.
Is scalp inflammation a chronic disease?
Scalp Inflammation with thinning of the hair and which may resemble psoriasis. Dermatomyositis is a chronic disease with no cure, however, many people respond well to certain treatments or combination of treatments with improvement in skin and muscle symptoms or even complete remission.
