Dermatomyositis: Suhani Bhatnagar, an actor who starred in the 2016 film Dangal, has succumbed to medical complications that arose while she was being treated for dermatomyositis, a rare autoimmune disease that causes muscle inflammation, at 19 years of age. On February 7, she was admitted to the All India Institute of Medical Sciences (AIIMS), Delhi because her entire body was swollen. She passed away on February 17.
Bhatnagar's father said that her disease was identified after tests were conducted at AIIMS. Her mother said that while receiving treatment, she contracted an infection due to which fluids were produced in her body. These fluids caused her lungs to collapse, and she eventually died, news agency ANI reported.
Here is everything you need to know about dermatomyositis.
The autoimmune disease results in skin rashes, along with chronic muscle inflammation. Dermatomyositis is similar to polymyositis, which is also an inflammatory disease that causes swelling, tissue damage, muscle weakness, and tenderness, but does not involve skin rash. They belong to a class of diseases called myopathies, which affect the skeletal muscle structure and metabolism, causing muscle weakness and pain.
Mostly, children aged five to 15 years, and adults aged 40 to 70 years develop dermatomyositis.
The likelihood of women developing dermatomyositis is twice that of men.
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Causes of dermatomyositis
Scientists have not yet found the exact cause of dermatomyositis. According to Johns Hopkins Medicine, possible causes of dermatomyositis include cancer, muscle infections, medications, environmental triggers, abnormal genes inherited from parents, or some other autoimmune disease.
People with a connective tissue disease such as lupus or rheumatoid arthritis can develop dermatomyositis.
Symptoms of dermatomyositis
The symptoms of dermatomyositis include difficulty swallowing, purple-red skin rash, itchy rashes, muscle weakness in the hip, shoulders, neck, and back, stiffness and pain in the joints, soreness, red- or purple-coloured upper eyelids, shortness of breath, swollen and red areas around fingernails, tiredness, fever, weight loss, trouble rising from a chair or getting out of bed, red or purple spots on the elbows, toes, knuckles and knees, scaly, rough and dry skin that can lead to hair thinning, hard lumps under the skin due to calcium deposits, voice changes, and muscle aches.
Skin rashes caused due to dermatomyositis are patchy, and red or purple in colour. These discolourations develop on the eyelids and on the muscles used to extend or straighten joints such as elbows, toes, knuckles, and knees.
There may also be red rashes on the face, shoulders, back, neck, and upper chest.
Sun exposure can exacerbate the rashes.
In some cases, muscle weakness can spread to the heart, gastrointestinal tract, and lungs, leading to coughing and difficulty in breathing. Adults with dermatomyositis may suffer from lung inflammation, sensitivity to light, weight loss, and low-grade fever, according to the US National Institutes of Health (NIH).
Both children and adults can have hard bumps under their skin or in the muscle due to calcium deposits. This condition, called calcinosis, mostly occurs one to three years after the onset of dermatomyositis, but may occur several years later in some cases.
Children are more likely to develop calcinosis than adults.
Sometimes, a person suffering from dermatomyositis may feel pain and weakness in the distal muscles, which are the muscles in the forearms and around the ankles and wrists.
Patients with this disease find difficulty climbing stairs, or raising their arms over their head.
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Diagnosis of dermatomyositis
Dermatomyositis can be diagnosed through blood tests that check levels of muscle enzymes called creatine phosphokinase and aldolase. The levels of these enzymes increase in patients with dermatomyositis.
Other ways to detect dermatomyositis include electrocardiogram and electromyography, which measures muscle response or electrical activity in response to a nerve's stimulation of the muscle.
Magnetic resonance imaging, skin and muscle biopsies, chest X-ray and commuted tomography scans, swallowing studies, cancer screening tests, lung function tests, and examination of myositis specific and associated autoantibodies are other ways of diagnosing dermatomyositis.
Blood tests search for signs of muscle inflammation, and abnormal proteins that form in autoimmune diseases. They are also used to detect antinuclear antibodies, which are autoantibodies that bind to the contents of the cell nucleus.
Magnetic resonance imaging detects inflammation in the body, and skin and muscle biopsies search for proteins by looking at tissues under a microscope.
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Treatment of dermatomyositis
The disease cannot be cured, but symptoms can be controlled.
Dermatomyositis is treated using corticosteroid medicines because they control inflammation, increase muscle strength, and relieve the patient of pain. As muscle strength improves, the dose of medicine is reduced. It takes about four to six weeks for one's condition to improve.
Immunosuppressant drugs such as azathioprine, methotrexate, cyclosporine A, tacrolimus, mycophenolate, and cyclophosphamide can be used instead of corticosteroids.
If there is no improvement despite using corticosteroids and immunosuppressants, biologic drugs or intravenous gamma globulin injections are administered to ease inflammation. Intravenous immunoglobulins boost one's immune system.
Physical therapy can help strengthen the muscles.
Dermatomyositis patients must wear sunscreen to prevent skin rashes. Some may be prescribed antihistamine drugs or anti-inflammatory steroid creams to treat itchy skin rashes.
Once conditions improve and muscles become stronger, the dose is reduced. Some patients with dermatomyositis consume prednisone all throughout their lives.
When a tumour is responsible for dermatomyositis, surgically removing it can reduce muscle weakness and rash. Surgery can also be conducted to remove calcium deposits.
Complications associated with dermatomyositis
Lung disease, cancer, joint pain, acute renal failure, and heart inflammation are some complications that may arise in dermatomyositis patients.
Dermatomyositis can lead to disability or be fatal if it causes severe illness.
The chances of a dermatomyositis patient dying are greater when they have severe muscle weakness, pneumonia, malnutrition, lung failure, or cancer.
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