Dermatomyositis (patient information)

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 * Associate Editor-In-Chief: Jinhui Wu, MD, Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]

Overview
Dermatomyositis is an uncommon inflammatory myopathy accompanied with a skin rash. The cause is unknown and researches suggest that it is associated with autoimmune disorders. Common signs and symptoms of dermatomyositis include difficulty swallowing, muscle weakness, purple-red skin rash, shortness of breath, fatigue and weight loss. Electromyography, even muscle biopsy or skin biopsy may help diagnose dermatomyositis. There is no cure for dermatomyositis. Medications and physical therapy may help relieve symptoms and maintain muscle strength. Prognosis of dermatomyositis varies from person to person. It depends on whether the patient appear severe complications.

What are the symptoms of Dermatomyositis ?
The most common signs and symptoms of dermatomyositis include:
 * Difficulty swallowing
 * Muscle weakness, stiffness, or soreness, appearing suddenly or developing slowly over weeks or months.
 * Purple or violet colored upper eyelids
 * Purple-red skin rash over the face, knuckles, neck, shoulders, upper chest, and back.
 * Shortness of breath
 * Fatigue and weight loss
 * Fever
 * Gastrointestinal ulcers and intestinal perforations in children

Diseases that can present with similar symptoms are
 * Hypothyroidism
 * Sarcoidosis

What causes Dermatomyositis?
Its an autoimmune disease who's actual cause is unknown.

Who is at highest risk?
The cause of dermatomyositis is unknown. Its more commonly found in women in extremes of age-group. Also presence of one connective tissue disorder or any other cancer makes you prone for it.

When to seek urgent medical care?
Call your health care provider if symptoms of dermatomyositis develop.

Diagnosis

 * Medical history and physical examination
 * Blood test: Blood tests such as creatine kinase (CK) and aldolase may help diagnose dematomyositis. Patients with dematomyositis may show increased CK and aldolase levels, indicating muscle damage.
 * Electromyography: During this procedure, the doctor inserts a thin needle electrode into the muscle and measure electrical activities when the patient relaxs or tightens the muscle.The doctor can identify a muscle disease by analysing these electrical activities.
 * Muscle biopsy: During this procedure, the doctor removes a small piece of muscle tissue surgically and detects in the pathological lab. Under microscope, tissues of patients with dermatomyositis may demonstrate that inflammatory cells surround and damage the capillary blood vessels in the muscle.
 * Muscle MRI: This image can detect subtle muscle inflammation and swelling early in the disease.
 * Skin biopsy: The goal of skin biopsy is to identify the diagnosis of dermatomyositis and rule out other disease such as systemic lupus erythematosus. During this procedure, the doctor removes a small piece of skin tissue surgically and detects in the pathological lab.

Treatment options
There is no cure for dermatomyositis. The goal of treatment is to relieve symptoms.
 * General treatment
 * Keep a regular exercise to maintain your muscle strength.
 * Bedrest when you feel tied.
 * Avoid exposure to sunshine: Use suncream and protective clothing may prevent your rash worse.


 * Medications
 * Corticosteroids: Corticosteroids can decrease swelling and inflammation by suppressing the immune system. Side effects of corticosteroids may include a decreased ability against infection, worse healing in the wound and osteoporosis.
 * Immunosuppressants: Immunosuppressive drugs, such as cyclophosphamide, mycophenolate mofetil, or azathioprine, may be used if the patient has an inadequate response or excessive sensitivity to corticosteroids.
 * Cytotoxic drugs: These kind of drugs may interfere with growth of normal and neoplastic cells by cross-linking of DNA or RNA or proteins and can improve signs and symptoms of dermatomyositis. Usual drugs include cyclophosphamide and azathioprine. Side effects include marrow suppression, liver damage, nausea and vomiting.
 * Intravenous immunoglobulin (IVIg): High dose of immunoglobulin can inhibit the damaging antibodies to attack muscle and skin in dermatomyositis.
 * Antimalarial medications: These type of drugs, such as hydroxychloroquine and chloroquine, can be used to trear a persistent rash.


 * Physical therapy: The physical therapist can make an exercise plan to help you maintain and improve muscle strength and flexibility.
 * Surgery: Surgery may be used to remove local areas of calcinosis.

Where to find medical care for Dermatomyositis?
Directions to Hospitals Treating dermatomyositis

What to expect (Outlook/Prognosis)?
Prognosis of dermatomyositis varies from person to person. Some children with dermatomyositis may recover, while other adults may die of complications. It depends on:
 * Therapy respond of the patient.
 * Whether the patient appear complications, such as acute renal failure, cancer, inflammation of the heart, joint pain or lung disease.

Possible complications
It can have various complication depending upon the system involvement
 * Muscular Involvement present as difficulty swallowing, aspiration pneumonia,
 * Skin Involvement present as infections and calcinosis