Dermatomyositis Treatment in Ankara, Turkey

Dermatomyositis is a rare autoimmune disease that affects both the skin and skeletal muscles, causing inflammation as the immune system targets microscopic vascular networks and muscle tissue. This systemic disorder, which belongs to the group of idiopathic inflammatory myopathies, is based on the principle that the body’s defense mechanism mistakenly perceives its own healthy tissues as foreign substances and attacks them. The clinical picture is defined by progressive weakness in proximal muscles and characteristic photosensitive skin lesions, and when not treated early, the process may result in systemic tissue damage that limits functional capacity.

In Whom and at What Ages Is Dermatomyositis Seen?

When we look at the distribution of this disease in society, we see that it is actually not very common and belongs to the group of rare diseases. However, being rare does not mean that it is insignificant. Although statistics tell us that only a few people out of every hundred thousand receive this diagnosis each year, we know that the risk increases in certain groups. In particular, the immune system of women shows a slightly higher tendency toward autoimmune diseases compared to men. This rule also applies to dermatomyositis, and our female patients are approximately twice as common as our male patients.

The relationship of the disease with age also draws a very interesting graph. In medicine we call this a “bimodal distribution,” meaning the disease peaks during two different periods of life.

The age groups in which the disease is most frequently seen are:

• Children between the ages of 5 and 15
• Adults between the ages of 40 and 60

The type seen in children is called “Juvenile Dermatomyositis,” and its course can be somewhat different from that in adults. In adults, it usually appears around middle age, during one of the most active periods of life. Can the disease occur outside these two periods? Of course it can, but these are the age ranges where we encounter it most frequently.

What Causes Trigger the Development of This Disease?

This is undoubtedly the question our patients ask us most often and are most curious about: “Why me? Why now?” In fact, there is no single and simple answer to this question. Dermatomyositis is like a genetic lottery ticket; you may carry this ticket in your pocket (meaning you have a genetic predisposition), but unless the draw occurs (meaning there is an environmental trigger), the disease does not appear.

Scientific research points to certain culprits that disrupt the immune system. Viral infections are among the leading ones. Especially some viruses experienced during childhood or agents such as the Epstein-Barr virus, which is common in society, can start this process by excessively stimulating the immune system. While the system tries to eliminate the virus, the braking mechanism fails and the attack turns toward our own tissues.

Another and perhaps the most important trigger is sunlight. Ultraviolet rays cause structural changes in skin cells, drawing the attention of the immune system to that area. In the stories of many of our patients, we hear that complaints began after an intense summer holiday or sunburn. In addition, some medications also appear on our list. Especially certain groups of drugs known as cholesterol-lowering medications or agents used in cancer treatment may rarely trigger this condition.

The main suspected triggers are:

• Genetic predisposition
• Viral infections
• Intense sun exposure
• Certain groups of medications
• Periods of intense stress

What Are the Skin Symptoms Seen in Dermatomyositis?

In the diagnosis of dermatomyositis, the language of the skin is our most reliable guide. Most of the time, the skin begins to give signals before the patient even notices muscle weakness. These signals are so characteristic that we call them “pathognomonic,” meaning findings that are specific only to this disease.

The first area that usually draws our attention is the face. The unique color change that appears on the eyelids, especially the upper eyelid, is almost the calling card of the disease. A reddish discoloration in violet, lilac, or light purple tones occurs. Medically we call this the “Heliotrope rash.” It takes its name from the purple heliotrope flower that turns toward the sun. This purple discoloration is often accompanied by mild swelling of the eyelids. When you wake up in the morning, you may encounter swelling in your eyes and a look as if you have applied purple eyeshadow.

The hands are another important source of clues. We ask you to look carefully at the knuckles that appear when you make a fist. On these joint areas on the backs of the fingers, small bumps of a purplish-red color, sometimes slightly scaly, appear. We call these “Gottron’s Papules.” Sometimes these red areas are not limited to the finger joints but can also appear as broader patches on the elbows or kneecaps. This is the finding most commonly confused with psoriasis or eczema, but its location is distinctive for us.

The regions where characteristic skin findings are located include:

• Upper eyelids
• Finger joints
• Outer surface of the elbows
• Kneecaps
• Neck and décolletage area
• Back and shoulders
• Outer sides of the thighs

What Signs Occur in Sun-Exposed Areas?

I mentioned the “love-hate” relationship this disease has with the sun. Sensitivity to light (photosensitivity) is part of the nature of this disease. Therefore, we see very typical rashes in areas not covered by clothing and directly exposed to sunlight.

Especially on the front part of the neck and the chest décolletage area, redness appears exactly in the shape of the letter “V.” We call this the “V sign.” It looks as if you have a sunburn, but it does not fade and over time the color darkens and vascularization increases. The same situation applies to the back. The rash that starts from the nape of the neck and spreads to the shoulders and upper back, appearing as if a shawl has been placed on the shoulders, is called the “Shawl sign.” Over time, the skin in these areas becomes thinner, capillary vessel dilations appear, and the color becomes mottled.

There is another interesting finding on the hands called “Mechanic’s Hands.” The sides and tips of the fingers become dry, hardened, calloused, and cracked. They resemble the worn hands of a worker dealing with oily or dirty work. This finding is very valuable for us because it is often an early warning that the disease may also involve the lungs. When we look at the nail beds, we notice that the cuticles are disrupted and the capillaries at the base of the nails dilate, forming red dots or lines that can even be seen with the naked eye. This microscopic vascular damage is the clearest indicator that the disease is active.

How Does Muscle Weakness Affect Daily Life?

Although skin findings are the visible aspect of the disease, the condition that most reduces the patient’s quality of life is muscle involvement. However, the weakness here is not like muscle pain experienced after sports. It is a more insidious and progressive process. Dermatomyositis targets the large muscle groups close to the trunk, which we call “proximal.” In other words, it affects the shoulder girdle and hip girdle muscles.

Patients often think this condition is simply fatigue. However, when we question it, very specific difficulties emerge. For example, while drying your hair you may notice that the arm holding the hair dryer becomes tired quickly and you have to lower it. Placing a plate on a high shelf in the kitchen may turn into torture. When the hip muscles are involved, getting up from a low sofa or toilet without support may become impossible. Climbing stairs or stepping up onto a bus step may no longer be as easy as before.

This weakness is usually symmetrical, meaning it affects both the right and left sides equally. Pain does not always have to be present. Some patients may have tenderness or aching in the muscles, but the main problem is the loss of “strength.” The difficulty is more noticeable during movements performed against gravity rather than while walking or carrying objects. If left untreated, this condition may progress to the point where the patient becomes bedridden.

The activities in which muscle weakness is most commonly felt include:

• Combing hair
• Drying hair
• Reaching for a high shelf
• Climbing stairs
• Standing up from a seated position
• Stepping onto a curb
• Lifting the head from the pillow

What Problems Can Occur in the Body’s Internal Systems?

Dermatomyositis is not only a disease of the skin or muscles; it is a systemic inflammation. This inflammation can sometimes spread to internal organs. The organ we worry about the most and monitor closely is the lungs. “Interstitial Lung Disease,” characterized by stiffness and inflammation of lung tissue, may occur in some of our patients. At first there may only be mild shortness of breath during exertion or a dry, irritating cough. However, if neglected it can progress to respiratory failure. We monitor our patients who show the “Mechanic’s Hands” sign much more closely in this respect.

Another important risk area is swallowing function. The act of swallowing also occurs thanks to muscles. Weakness of the muscles in the upper part of the esophagus and throat can make swallowing food difficult. The patient may feel food getting stuck when swallowing solid foods or liquids may go up into the nasal passages. This condition not only leads to nutritional problems but also creates the risk of pneumonia (aspiration pneumonia) if food enters the lungs.

Although heart involvement is rarer, it may appear as inflammation of the heart muscle or rhythm disturbances. In the joints, pains and swelling similar to rheumatoid arthritis may occur, but they usually do not leave permanent damage or deformity.

Is There a Relationship Between Dermatomyositis and Cancer?

This is a reality that we are most sensitive about but must honestly share when talking with our patients. Especially dermatomyositis that appears in adults (over the age of 40) may sometimes be a sign of a hidden tumor in the body. We call this a “paraneoplastic syndrome.” In other words, while the immune system is actually trying to recognize and attack cancer cells, it may mistakenly attack muscle and skin cells as well.

Approximately one quarter of adult patients may have an underlying malignancy (cancer). This risk is highest in the first year when the disease is diagnosed and continues for the following 3–5 years. Therefore, every adult patient we diagnose with dermatomyositis undergoes a detailed cancer screening from head to toe. This screening should not frighten our patients; on the contrary, thanks to this disease it may be possible to detect a cancer that has not yet produced any symptoms at a very early stage and treat it with complete recovery.

The main cancer types that should be screened in women and men include:

• Ovarian cancer
• Breast cancer
• Lung cancer
• Colon cancer
• Stomach cancer
• Lymphoma
• Prostate cancer

How Is the Disease Diagnosed and With Which Tests?

The diagnostic process is like assembling the pieces of a puzzle. No single test tells us “yes, this is dermatomyositis.” We reach the conclusion by combining clinical observation, laboratory data, and imaging methods.

In the first step we look at blood tests. When muscle fibers break down, certain enzymes mix into the blood. One of the most important of these is “Creatine Kinase (CK).” The level of CK shows us the extent of muscle damage. In addition, increases may also be seen in liver tests such as AST, ALT, and LDH; this can sometimes cause confusion by appearing to be liver disease, but in fact the source is the muscles.

One of the greatest opportunities modern medicine offers us is the “Myositis-Specific Antibodies (MSAs)” panel. With this test we search for specific fighter molecules in the patient’s blood. Which antibody is positive helps us predict the course of the disease. For example, if the “Anti-Mi2” antibody is positive, the patient usually responds very well to treatment and the cancer risk is low. However, if “Anti-TIF1-gamma” is positive, we keep cancer screening much stricter. In the presence of “Anti-Jo1,” we monitor the lungs more closely.

Other auxiliary methods used in diagnosis include Electromyography (EMG) and Magnetic Resonance Imaging (MRI). With EMG we examine the electrical activity of the muscles and distinguish whether the problem originates in the nerve or the muscle. With MRI we visualize inflammation and edema in the muscles. For a definitive diagnosis, skin or muscle biopsy is usually required. A small piece taken from the affected area is examined under a microscope, allowing the disease to be identified.

The main tools used in the diagnostic process include:

• Physical examination
• Muscle enzyme test
• Autoantibody panel
• Electromyography
• Muscle MRI
• Skin biopsy
• Muscle biopsy

How Is Dermatomyositis Treated?

The treatment process is a long marathon in which the patient and the doctor work hand in hand. Our goal is not only to extinguish the inflammation but also to prevent it from flaring up again and to maintain the patient’s quality of life at the highest level. The treatment plan is tailored individually for each patient.

The first and most important step of treatment is not medications but lifestyle changes. Protection from the sun is an indispensable rule of treatment. Even if you use the most expensive medications, it is very difficult to eliminate skin findings if you do not protect yourself from the sun. Therefore we advise our patients to use sunscreen even in winter.

In medication treatment, the gold standard is “corticosteroids,” in other words cortisone. Cortisone is the most effective firefighter that rapidly suppresses the aggressive attack in the immune system. It is started at a high dose and gradually reduced little by little over months as the inflammation comes under control. However, because we know the long-term side effects of cortisone (weight gain, high blood pressure, osteoporosis), we always add a second medication that provides “steroid-sparing” effects.

This second group of medications (Methotrexate, Azathioprine, Mycophenolate, etc.) regulates the immune system in a more balanced way and over time allows us to completely discontinue cortisone. In patients who have only skin findings, medications such as “Hydroxychloroquine,” which is used in malaria treatment but which we love very much in dermatology, are also quite effective. In very resistant or severe cases, intravenous immunoglobulin treatments (IVIG) or smart molecules (biological agents) come into play.

What Should Patients Pay Attention to in Daily Life?

Receiving a diagnosis of dermatomyositis does not mean life is over; it only means that a new routine must be established. How you care for your body is just as important as taking your medications regularly.

In terms of nutrition, during periods when you use cortisone you need to remove salt from your life. Salt causes the body to retain water and increases blood pressure. In addition, eating a protein-rich diet (eggs, fish, legumes) helps muscles repair themselves. Taking calcium and vitamin D supplements to reduce the risk of osteoporosis should not be neglected.

Exercise is a delicate balance. During periods when the disease is very active and the muscles are very weak, we avoid heavy sports because it can increase muscle breakdown. Passive stretching movements are sufficient during this period. However, once inflammation is suppressed, regular exercise is necessary to regain muscle strength. Inactivity causes muscles to waste away more quickly. A program performed under the supervision of a physiotherapist is the most appropriate.

The lifestyle recommendations that should be followed include:

• High-protection sunscreen
• Wide-brimmed hat
• Low-salt diet
• Protein-rich nutrition
• Regular exercise
• Vitamin D supplementation
• Avoiding smoking

How Long Does Treatment Take and Is Recovery Possible?

The biggest concern of our patients is the question: “Will this disease ever go away?” Dermatomyositis is a chronic disease, meaning it requires long-term follow-up like diabetes or hypertension. However, this does not mean you will spend your life in bed or live with rashes.

The treatment process usually lasts 1–2 years or longer. Our goal is to bring the patient into what we call “remission,” a phase in which the disease is completely asleep, symptoms disappear, and the need for medication is minimized (or eliminated entirely). Many of our patients, with the right treatment and a compatible lifestyle, can bring the disease completely under control and continue their normal lives.