dermatomyositis

(dĕr″măt-ō-mī″ŏ-sīt′ĭs)

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[dermato- + myositis]
A rare acute, subacute, or chronic disease of connective tissue, of unknown cause, marked by edema, rash, weakness, pain, and inflammation of the muscles.
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DERMATOMYOSITIS

SYMPTOMS
Symptoms include fever, malaise, and weakness (esp. of the pelvic and shoulder girdle muscles, neck, and pharynx), lesions of the skin and mucosa, e.g., Gottron papules, and discomfort in the joints. Performance of ordinary activities (such as getting up from a chair, combing one's hair, reaching over one's head, raising one's head from a pillow) are affected. About one third of patients have dysphagia. Dysphonia and difficulty in breathing also may be present. Associated respiratory disease, heart failure, cancers, or adverse effects of drug therapies may hasten death. The disease progresses slowly, with frequent exacerbations and remissions, and occurs twice as often in females than in males. The prognosis worsens with age.

TREATMENT
The treatment is symptomatic and includes bedrest, physical therapy, high doses of steroids, and other anti-inflammatory agents. Cytotoxic drugs such as azathioprine, cyclophosphamide, and methotrexate often help patients who do not respond to adrenocortical steroids. Analgesics are provided as necessary. Serum muscle enzyme levels and muscle strength usually improve after 2 to 6 weeks of aggressive therapy, allowing corticosteroid dosages to be gradually tapered down.

PATIENT CARE
The patient's level of discomfort, muscle weakness, and joint range of motion (ROM) are assessed and documented daily. The patient's face, neck, upper back, chest, nail beds, eyelids, and interphalangeal joints are evaluated for rashes, and findings are documented. Frequent assistance is provided to help the patient reposition in correct body alignment; appropriate supportive devices, frequent passive ROM exercises, and graduated exercises are used to prevent and treat muscle atrophy and joint contractures. Warm baths, moist heat, and massage are provided to relieve stiffness, and prescribed analgesics are administered. Oral lesions are irrigated with warm saline solution as necessary. Tepid sponge baths and compresses are used to relieve pruritus and to prevent scratching; antihistamines are also administered as prescribed. Self-care activities, with assistance if necessary, are encouraged and paced according to the patient's response. Reassuring the patient that muscle weakness during exacerbations is temporary helps to ease fears of dependence. Prescribed corticosteroid, cytotoxic, or immunosuppressant drugs are administered, and the patient's response is evaluated.

Both patient and family are educated about the progress of the disease, expectations from treatment, and possible adverse reactions to corticosteroid, cytotoxic, and immunosuppressant therapies. Good nutrition and a low-sodium diet are recommended to prevent fluid retention. The patient should be educated about the potential side effects of therapy (such as those associated with chronic, high-dose corticosteroids) and helped to develop physical and emotional coping skills to deal with these. The patient is encouraged to express feelings, fears, and concerns about the illness. Realistic support and encouragement are provided.

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DERMATOMYOSITIS

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