Dermatomyositis

Dermatomyositis is an inflammatory myopathy in which the muscles get inflamed. This disorder affects usually the skin and muscles but in some cases it can also affect joints, lungs, and esophagus or may be heart. The most common symptom of this inflammatory disease is a weakness in muscles.

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It is a not so common inflammatory disease characterized by the weakness of muscle and skin rash. It can occur both among the children as well as adults. The adults age group affected by this disease is 40s to 60s and among children the age groups varies between 4 to 16 years. Generally dermatomyositis occurs in females and less commonly in males.

There are treatments which can clear the skin rash fully and can help regain muscle strength.

Signs and symptoms

There are many signs and symptoms of dermatomyositis which can help you identify this skin disorder. In almost 40% of the individuals, the onset of dermatomyositis is accompanied by skin disease. Following are the symptoms of cutaneous or beginning of dermatomyositis:

  • Scaly scalp or hair loss
  • Pruritis of skin lesions
  • Eruption on exposed skin

Dermatomyositis is associated with problem in muscles. The muscle disease may precede or follow the skin disease. Following are the muscle problems related to the condition-

  • Proximal muscle weakness
  • Sometimes muscle tenderness may occur
  • Muscle fatigue particularly when walking, combing hair, climbing stairs, standing after sitting for long etc.

Along with the above mentioned problems, dermatomyositis also includes systematic symptoms such as:

  • Dyspnea
  • Dysphagia
  • Dysphonia
  • Arthralgia
  • Arrhythmia
  • Malignancy

Children with dermatomyositis also develop flexion contracture in ankles and extra muscular manifestations including:

  • Dysphagia which is much similar to scleroderma
  • General problems like fever, weight loss, malaise, arthralgia and Reynaud phenomenon.
  • Gastrointestinal ulcers and infections
  • Contracture joints
  • Pulmonary and interstitial lung disease
  • Atriventricular problems, dilated cardiomyopathies and tachyarrhythmia
  • Subcutaneous calcification

Other symptoms of dermatomyositis that occur in almost everyone suffering from this skin disease include following:

  • Difficulty in swallowing
  • Fatigue and weight loss
  • Fever
  • Muscle pain and tenderness
  • Problems in lungs
  • Calcinosis i.e. hardened deposit of calcium underneath the skin.
  • Gastrointestinal ulcers and perforations in intestine which is most common among children.

Diagnosis

To diagnose if the person is suffering from cutaneous dermatomyositis following examinations are done:

  • Flat, red rash on face, upper trunk and rest parts of the body such as neck, anterior chest, knees, back and shoulders and elbows.
  • Pathognomonic cutaneous features including Gottron papules and Heliotrope.
  • Other features include periungual and cuticular changes, Malar erythema, violaceous erythema, violaceous erythema on extensor surfaces and poikiloderma in photosensitive distribution.

The extent of muscle involvement in dermatomyositis is examined through following:

  • Distal strength, tendon reflex or sensation.
  • Quadriparesis
  • Flexor muscles and extensor muscles are affected with extensor muscles usually getting affected more than flexor muscles.

Tests and Imaging studies for dermatomyositis

There are a number of tests for testing dermatomyositis. Some of the laboratory and other related studies include:

  • Electrocardiography
  • Antinuclear antibody levels
  • Studies related to pulmonary function
  • Myositis-specific antibodies
  • Studies to determine muscle enzyme levels

Imaging studies to detect dermatomyositis include the following: Magnetic Resonance Imaging, CT scan, Chest radiography, ultrasonography, electromyography and Barium shallow.

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In order to evaluate the symptoms of dermatomyositis, following procedures are available: Skin biopsy, muscle biopsy and esophageal manometry.

Prognosis

Though most patients survive, there is a chance of disability. Remission is evident in 22 percent of the cases. Children with severe condition may end up with contractures.  Patients require consistent care and therapy. Patients who have cardiac issues, pulmonary problems etc and are above 60 years of age, can have poor prognosis. Death is caused usually due to weakness in the muscles and cardiopulmonary problems.

Treatment

If dermatomyositis is caused due to muscle involvement, corticosteroids with or without immunosuppressive agent is helpful. Other things to manage this skin disease are avoiding exposure to sun, using sunscreens, antimalarial agents, topical corticosteroids, mycophenolate mofetil, mycophenolate mofetil, methotrexate and immunoglobulin.

Medications used to treat dermatomyositis are as follows:

  • Immune globulins
  • Antimalarial agents
  • Immunosuppressive agents such as methotrexate, sirolimus, mycophenolate modetil, rituximab and azathioprine.
  • Corticosteroids such Prednisone. Prednisone is the best-known medication for dermatomyositis.
  • Calcium channel blockers such as diltiazem
  • Colchicines, alendronate and warfarin are also used.

Some of the non-pharmacotherapy or general measures to treat dermatomyositis are as follows:

  • Avoiding exposure to sun
  • Using elevation for head rest while lying on bed
  • Spending fair time doing bed rest
  • Sun protection
  • Physical therapy and rehabilitative measures
  • Avoid eating before going to bed

There are also surgical procedures to manage dermatomyositis. Although surgery is not the only option but some patients prefer to take up surgery to remove local area of calcinosis.

When to consult a doctor

If you or your near and dear ones have developed signs and symptoms of dermatomyositis, it becomes important to see a doctor to evaluate the symptoms and diagnose the disease. And if already have been diagnosed with this skin disease and you witness some new symptoms developing then you should immediately consult your doctor.

Dermatomyositis pictures

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