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Dermatomyositis

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Dermatomyositis is an inflammatory myopathy that is characterized by muscle inflammation that causes muscle weakness. It is an idiopathic inflammatory myopathy which means that the cause of the disease is unknown. Because of this, a set of criteria to aid in its diagnosis was suggested by Bohan and Peter in 1975. This set of criteria is also the basis of classification for polymyositis and dermatomyositis. There were all in all 5 criteria that were suggested 4 of which are muscle disease related. The four criteria are elevated muscle enzymes, progressive proximal symmetric weakness, abnormal finding on the muscle biopsy sample, and an abnormal finding on electromyogram. The fifth and last criterion was compatible cutaneous disease.

DERMATOMYOSITIS SIGNS AND SYMPTOMS

It has been noted for a long time that dermatomyositis is associated with malignancy. It is a systematic disorder that usually affects the lungs and esophagus. It can also affect the heart in rare cases. Children and adolescents suffering from dermatomyositis may develop a complication called calcinosis.

Dermatomyositis is actually one in the group of five subsets of myositis. Each subset has its own unique characteristics that which is used to distinguish one from the other.

It is possible that a person suffering from dermatomyositis could die. This could be because of muscle weakness or an effect of the disease to the cardiopulmonary organs. If a patient is known to have developed malignancy, this too may be the cause of death. The prognosis for dermatomyositis however is good and the survivability rate is high for as long as other complications in other organs such as the heart or the lungs have not been diagnosed. Survivors could develop disability or residual weakness and for children, development of contractures is possible.

Dermatomyositis has no preference and can actually affect anyone regardless of race, sex and age. It has been known though that the disease frequently occurs more in females than in male.

The onset of dermatomyositis is usually manifested initially with the presence of skin disease. The muscle disease however may precede the skin disease, may occur together with the skin disease or may even occur from weeks to years following the manifestation of the skin disease.

The skin disease is characterized by being pruritic or very itchy. On exposed surfaces, eruptions may become noticeable. It may also be observed that the there is diffuse hair loss and that scalp has become scaly.

The involvement of muscle is initially manifested by weakness of muscles in the proximal area. Sufferers may observe muscle fatigue when doing normal activities such as walking, climbing stairs, reaching for items, or standing up from a sitting position. There is also chance for the patient to experience muscle tenderness in some, but rare cases. There may also be the presence of joint swelling with Raynaud phenomenon. Abnormalities in lab examinations of the heart can also signify the presence of the disease.
dermatomyositis
Image: Dermatomyositis


Manifestations of the disease dermatomyositis may also be systemic. The presence of arthritis, arthralgia, dysphagia, dyspnea, dysphonia, and arrhythmia are some of the systemic manifestations caused by dermatomyositis.

As have been mentioned, malignancy is often associated with dermatomyositis. This occurs however mostly to adults with ages 60 years or more. Malignancy occurs rarely in children.

Speaking of children, onset of the disease may be very subtle and can only be clearly observe with the onset of the skin disease. A symptom that is mostly inherent with children who have dermatomyositis is hard nodules of the skin – a symptom of calcinosis which is a complication of dermatomyositis in children.

DERMATOMYOSITIS DIAGNOSIS

During the clinical diagnosis of the disease, physicians may look for the cutaneous features of dermatomyositis which are Gottron papules and heliotrope rash. Other features include poikiloderma, and erythema. The heliotrope rash may or may not incur edema. This type of rash is one of the definitive indications of dermatomyositis since it rarely occurs in other disease. Poikiloderma on the other can occur on exposed skin such that of the arms. Its presence can be noticed to be in a V-shaped distribution on the back, upper chest, and anterior neck.

DERMATOMYOSITIS TREATMENT

The treatment of dermatomyositis focuses both on the muscle disease and skin disease which is done through therapy. Other than the therapy, other treatment may also be given for other complications and manifestations of the disease.

Generally, a patient with dermatomyositis is required to have bed rest especially those that have been observed to have severe muscle inflammation. Physical therapy is also given to prevent contractures that may cause other complications in the joints. If a patient is known to have dysphagia or difficulty in swallowing, eating before bedtime is discouraged. This kind of situation sometimes requires the need for NGT (nasogastric tube) feeding to increase caloric intake of the patient.

As for medications, the muscle disease is treated using corticosteroids. Prednisone is commonly given as an initial dose. But because of the possibility of toxic effects related to steroids, some make it a point to administer cytotoxic agent in the early course of therapy.


For the skin disease, patients will be asked to avoid the sun or any kind of strong light since most often than not, patients become photosensitive. A drug called mathotrexate is also noted to be very useful in treating the skin disease.

It is really quite difficult to say what can prevent dermatomyositis since it is an idiopathic disease. The best way to prevent this kind of disease, just like other idiopathic disease, is to live a healthy lifestyle. Observe proper diet as well as taking care of your body properly. Try to stray from unhealthy vices such as smoking and excessive alcohol drinking.

In the case of dermatomyositis, it would be best not to neglect the initial manifestations of the disease. This would include the onset of muscle weakness and skin disease. When these kinds of symptoms come up, going to the doctor should be the first priority. Early detection and treatment of this disease entails a better prognosis.
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