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Dermatomyositis

 

Dermatomyositis is a muscle disease that involves inflammation and a skin rash. It is a type of inflammatory myopathy.

Causes

The cause of dermatomyositis is unknown. Experts think it may be due to a viral infection of the muscles or a problem with the body's immune system. It may also occur in people who have cancer in the abdomen, lung, or other parts of the body.

Anyone can develop dermatomyositis. It most often occurs in children age 5 to 15 and adults age 40 to 60. Women develop this condition more often than men.

Polymyositis is a similar condition, but the symptoms do not include a skin rash.

Symptoms

 

Symptoms may include:

  • Muscle weakness, stiffness, or soreness
  • Problems swallowing
  • Purple color to the upper eyelids
  • Purple-red skin rash
  • Shortness of breath

The muscle weakness may come on suddenly or develop slowly over weeks or months. You may have trouble raising your arms over your head, getting up from a sitting position, and climbing stairs.

The rash may appear on your face, knuckles, neck, shoulders, upper chest, and back.

 

Exams and Tests

 

The health care provider will do a physical exam. Tests may include:

  • Bloods test to check levels of muscle enzymes called creatine phosphokinase and aldolase
  • Blood tests for autoimmune diseases
  • ECG
  • Electromyography
  • Magnetic resonance imaging (MRI)
  • Muscle biopsy
  • Skin biopsy
  • Other screening tests for cancer

 

Treatment

 

The disease is treated with anti-inflammatory medicines called corticosteroids. You may also take drugs to suppress the immune system such as methotrexate, azathioprine, and hydroxychloroquine to treat the skin rash.

If the condition does not respond to these medicines, other drugs that suppress the immune system may be tried.

When your muscles get stronger, your doctor may tell you to slowly cut back on your doses. Most people with this condition must take a medicine called prednisone for the rest of their lives.

If a cancer is causing the condition, the muscle weakness and rash may get better when the tumor is removed.

 

Outlook (Prognosis)

 

Symptoms may go away completely in some people, such as children.

The condition may be fatal in adults due to severe muscle weakness, malnutrition, pneumonia, or lung failure. The major causes of death with this condition are cancer and lung disease.

 

Possible Complications

 

Complications may include:

  • Acute renal failure
  • Cancer (malignancy)
  • Inflammation of the heart
  • Joint pain
  • Lung disease

 

When to Contact a Medical Professional

 

Call your health care provider if you have muscle weakness or other symptoms of this condition.

 

 

References

Jorizzo JL, Vleugels RA. In: Bolognia JL, Jorizzo JL, Schaffer JV, et al, eds. Dermatology. 3rd ed. Philadelphia, PA: Elsevier Mosby; 2012:chap 42.

 
  • Dermatomyositis, Gottron's papule

    Dermatomyositis, Gottron's papule - illustration

    Red, thickened, scaly skin over the knuckles (Gottron's sign) associated with dermatomyositis, an autoimmune disease of the skin and muscles.

    Dermatomyositis, Gottron's papule

    illustration

  • Dermatomyositis, Gottron's papules on the hand

    Dermatomyositis, Gottron's papules on the hand - illustration

    This violet-colored inflammation (erythema) over the knuckles is caused by Dermatomyositis. Other skin conditions produce more redness, while the color of this lesion is violet. There may also be inflammation in the muscle tissue.

    Dermatomyositis, Gottron's papules on the hand

    illustration

  • Dermatomyositis, heliotrope eyelids

    Dermatomyositis, heliotrope eyelids - illustration

    This photograph demonstrates the sign "heliotrope eyelids" in which the eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.

    Dermatomyositis, heliotrope eyelids

    illustration

  • Dermatomyositis on the legs

    Dermatomyositis on the legs - illustration

    The appearance of purple (violaceous) plaques on the knees may be associated with dermatomyositis. Typically, most lesions associated with other diseases are red (erythematous).

    Dermatomyositis on the legs

    illustration

  • Dermatomyositis, Gottron's papule

    Dermatomyositis, Gottron's papule - illustration

    This is Gottron's sign, seen in dermatomyositis (an inflammatory disease of the muscles and skin). Violet-colored inflammation over the knuckles is an important diagnostic finding in dermatomyositis, since other skin conditions produce more redness.

    Dermatomyositis, Gottron's papule

    illustration

  • Paronychia, candidial

    Paronychia, candidial - illustration

    Candida paronychia produced periungual erythema, edema and nail fold maceration.

    Paronychia, candidial

    illustration

  • Dermatomyositis, heliotrope rash on the face

    Dermatomyositis, heliotrope rash on the face - illustration

    Dermatomyositis, a connective tissue disease, typically produces a reddish-purple (violaceous) rash. The rash is named after the tendency of plants to grow toward the sun (heliotropic) and is characteristic of dermatomyositis.

    Dermatomyositis, heliotrope rash on the face

    illustration

    • Dermatomyositis, Gottron's papule

      Dermatomyositis, Gottron's papule - illustration

      Red, thickened, scaly skin over the knuckles (Gottron's sign) associated with dermatomyositis, an autoimmune disease of the skin and muscles.

      Dermatomyositis, Gottron's papule

      illustration

    • Dermatomyositis, Gottron's papules on the hand

      Dermatomyositis, Gottron's papules on the hand - illustration

      This violet-colored inflammation (erythema) over the knuckles is caused by Dermatomyositis. Other skin conditions produce more redness, while the color of this lesion is violet. There may also be inflammation in the muscle tissue.

      Dermatomyositis, Gottron's papules on the hand

      illustration

    • Dermatomyositis, heliotrope eyelids

      Dermatomyositis, heliotrope eyelids - illustration

      This photograph demonstrates the sign "heliotrope eyelids" in which the eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.

      Dermatomyositis, heliotrope eyelids

      illustration

    • Dermatomyositis on the legs

      Dermatomyositis on the legs - illustration

      The appearance of purple (violaceous) plaques on the knees may be associated with dermatomyositis. Typically, most lesions associated with other diseases are red (erythematous).

      Dermatomyositis on the legs

      illustration

    • Dermatomyositis, Gottron's papule

      Dermatomyositis, Gottron's papule - illustration

      This is Gottron's sign, seen in dermatomyositis (an inflammatory disease of the muscles and skin). Violet-colored inflammation over the knuckles is an important diagnostic finding in dermatomyositis, since other skin conditions produce more redness.

      Dermatomyositis, Gottron's papule

      illustration

    • Paronychia, candidial

      Paronychia, candidial - illustration

      Candida paronychia produced periungual erythema, edema and nail fold maceration.

      Paronychia, candidial

      illustration

    • Dermatomyositis, heliotrope rash on the face

      Dermatomyositis, heliotrope rash on the face - illustration

      Dermatomyositis, a connective tissue disease, typically produces a reddish-purple (violaceous) rash. The rash is named after the tendency of plants to grow toward the sun (heliotropic) and is characteristic of dermatomyositis.

      Dermatomyositis, heliotrope rash on the face

      illustration

    Tests for Dermatomyositis

     

       

      Review Date: 1/20/2015

      Reviewed By: Gordon A. Starkebaum, MD, professor of medicine, division of rheumatology, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

      The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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