Heliotrope Rash and Other Dermatomyositis Symptoms

Heliotrope rash is caused by dermatomyositis (DM), a rare connective tissue disease. People with this disease have a violet or bluish-purple rash that develops on areas of the skin. They can also experience muscle weakness, fever, and joint aches.

The rash may be itchy or cause a burning sensation. It commonly appears on sun- exposed areas of the skin, including the:

  • face (including eyelids)
    • neck
  • knuckles
  • elbows
  • chest
    • back
  • knees
  • shoulders
    • hips
  • nails

It isn’t uncommon for a person with this condition to have purple eyelids. The purple pattern on the eyelids may resemble a heliotropeflower, which has small purple petals.

DM is rare. In the United States, researchers believe there are up to 10 cases per 1 million adults. Likewise, there are about three cases per 1 million children. Women are more commonly affected than men, and African-Americans are more commonly affected than Caucasians.

What causes heliotrope rash?

The rash is a complication of DM. This connective tissue disorder has no known cause. Researchers are trying to understand who is likely to develop the disorder and what increases their risk.

Possible causes of dermatomyositis include:

  • Family or genetic history: If someone in your family has the disease, your risk may be higher.
  • An autoimmune disease: A functioning immune system attacks unhealthy or invading bacteria. In some people, however, the immune system attacks healthy cells. When this happens, the body responds by causing unexplained symptoms.
  • Underlying cancer: People with DM are at a higher risk for developing cancer, so researchers are investigating whether cancer genes play a role in who develops the disorder.
  • Infection or exposure: It’s possible that exposure to a toxin or trigger could play a role in who develops DM and who doesn’t. Likewise, a previous infection may also affect your risk.
  • Complication of medication: Side effects from some medications could lead to a rare complication like DM.

Other symptoms of dermatomyositis

A heliotrope rash is often the first sign of DM, but the disease can cause other symptoms.

These include:

  • ragged cuticles that expose blood vessels at the nail bed
  • scaly scalp, which may look like dandruff
  • thinning hair
  • pale, thin skin that can be red and irritated

Over time, DM can cause muscle weakness and lack of muscle control.

Less commonly, people may experience:

  • gastrointestinal symptoms
  • heart symptoms
  • lung symptoms

Who’s at risk for heliotrope rash and dermatomyositis?

Currently, researchers don’t have a clear understanding of what factors may affect the disorder and rash. People of any race, age, or sex may develop the rash, as well as DM.

However, DM is twice as common in women, and the average age of onset is 50 to 70. In children, DM commonly develops between the ages of 5 and 15.

DM is a risk factor for other conditions. That means having the disorder may increase your odds for developing other conditions.

These include:

  • Cancer: Having DM increases your risk for cancer. People with DM are three to eight times more likely to develop cancer than the general population.
  • Other tissue diseases: DM is part of a group of connective tissue disorders. Having one may increase your risk for developing another.
  • Lung disorders: These disorders can eventually impact your lungs. You may develop shortness of breath or coughing. According to one study, 35 to 40 percent of people with this disorder develop interstitial lung disease.

How are heliotrope rash and dermatomyositis diagnosed?

If you develop a purplish rash or any other unusual symptoms, you should consult your doctor.

If your doctor suspects your rash is the result of DM, they may use one or more tests to understand what’s causing your issues.

These tests include:

  • Blood analysis: Blood tests can check for elevated levels of enzymes or antibodies that may signal potential problems.
  • Tissue biopsy: Your doctor may take a sample of muscle or the skin affected by the rash to check for signs of disease.
  • Imaging tests: An X-ray or MRI can help your doctor visualize what’s happening inside your body. This may rule out some possible causes.
  • Cancer screening: People with this disorder are more likely to develop cancer. Your doctor may perform a full-body exam and broad testing to check for cancer.

How is this rash treated?

As with many conditions, early diagnosis is key. If the skin rash is diagnosed early, treatment can begin. Early treatment reduces the risk of advanced symptoms or complications.

Treatments for heliotrope rash include:

  • Antimalarials: These medications can help with the rashes associated with DM.
  • Sunscreen: Exposure to sun may make the rash irritated. That may make symptoms worse. Sunscreen can protect the delicate skin.
  • Oral corticosteroids: Prednisone (Deltasone) is most often prescribed for heliotrope rash, but others are available.
  • Immunosuppressants and biologics: Medicines such as methotrexate and mycophenolate may help people with heliotrope rash and DM. That’s because these medicines often work to stop the immune system from attacking your body’s healthy cells.

As DM worsens, you may experience greater difficulty with muscle movement and strength. Physical therapy can help you regain strength and relearn functions.

Outlook

For some people, DM resolves entirely and all of the symptoms disappear, too. However, that’s not the case for everyone.

You may have symptoms of heliotrope rash and complications from DM for the remainder of your life. Adjusting to life with these conditions is made easier with proper treatment and watchful monitoring.

Symptoms of both conditions may come and go. You may have long periods during which you have no problems with your skin, and you regain almost-normal muscle function. Then, you may go through a period where your symptoms are much worse or more troublesome than before.

Working with your doctor will help you anticipate future changes. Your doctor can also help you learn to care for your body and your skin during inactive times. That way, you may have fewer symptoms or be more prepared during the next active phase.

Can this be prevented?

Researchers don’t understand what causes a person to develop heliotrope rash or DM, so steps for possible prevention aren’t clear. Tell your doctor whether you have a family member diagnosed with DM or another connective tissue disorder. This will allow the two of you to watch for early signs or symptoms so you can begin treatment right away if it’s ever necessary.

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