Heliotrope Rash Explained

Medically Reviewed By Meredith Goodwin, MD, FAAFP
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A heliotrope rash is a reddish-purple rash that appears on the upper eyelids. Along with the red or purple color, the eyelids can become swollen. The sudden appearance of a heliotrope rash is usually the first sign of an inflammatory muscle disorder called dermatomyositis.  Doctors named the rash after the heliotrope flower, which has a vibrant purple color. There are tests that confirm dermatomyositis, along with treatments that can help it. There is no cure for dermatomyositis, and other signs of the disease usually occur after the heliotrope rash appears. 

This article will explain heliotrope rash symptoms, their causes, and treatment for the rash and dermatomyositis. 

What is a heliotrope rash?

A man and his eye
Marie Cristabern Joyce Villamor/EyeEm/Getty Images

A heliotrope rash is a rash that appears on the upper eyelids. It is reddish-purplish in appearance and often makes the eyelids swell. Typically, the rash will affect both eyelids, but in some cases, it can only affect one eyelid. The rash may also look like a butterfly on the bridge of the nose and the cheeks. 

While it is possible to have other things that can lead to swollen eyelids, the main cause of a heliotrope rash is dermatomyositis, which is a rare inflammatory muscle disease. 

Read more about dermatomyositis here.

Is it an autoimmune condition? 

The exact cause of dermatomyositis is unknown, but doctors do think the immune system is involved. Doctors consider it to be an autoimmune disorder, so the immune system attacks otherwise healthy cells, like the muscles and skin. 

A combination of genetic predisposition, environmental triggers — and in some cases, cancer — may all lead to the development of dermatomyositis. The condition can affect anyone from birth to age 80, but more often occurs in adults between the ages of 40 and 60. 

Dermatomyositis is rare and occurs only in 9.6 out of every million people.

Read more about autoimmune disorders here.

What are the symptoms of heliotrope rash?

A heliotrope rash commonly appears as swollen, reddish-purple discoloration on the eyelids and cheeks.

The primary symptoms of a heliotrope rash are:

  • purple-red rash along the upper eyelids
  • eyelids may be swollen
  • usually affects both eyelids

A heliotrope rash may also appear on other areas of the skin. It most commonly occurs in areas of the skin that have exposure to the sun. These include your:

  • face
  • neck
  • shoulders
  • upper chest
  • back

Accompanying symptoms

If the heliotrope rash is a sign of dermatomyositis, it is usually the first symptom to appear. After the skin rash appears, other symptoms may follow. 

Progressive symptoms of dermatomyositis can include

  • pale, thinned skin with visible blood vessels and dark spots, known as poikiloderma
  • yellow-white bumps under the skin, known as calcinosis
  • worsening muscle weakness in the hips, thighs, shoulders, upper arms, and neck
  • extreme tiredness
  • difficulty completing tasks of daily life, such as combing your hair
  • weak, tender muscles
  • skin scaling
  • skin swelling
  • nail changes

What causes a heliotrope rash? 

A heliotrope rash is typically the first sign of dermatomyositis, a degenerative disease that affects the muscles and skin. Doctors are not entirely sure why a heliotrope rash develops, but think that it may link with the inflammatory changes that happen in the skin and muscles as a result of the disorder. 

Dermatomyositis is an inflammatory disease, so it can lead to swelling and skin changes. Doctors are uncertain as to what causes dermatomyositis to develop, but there are some known risk factors and triggers for the disease. 


The condition affects both children and adults but is more common in adults. It affects all genders equally in childhood. Dermatomyositis occurs in twice as many females as males. Researchers do know that certain drugs, cancers, and viruses link with dermatomyositis. 

Some pathogens researchers think may link with dermatomyositis are:

  • coxsackie virus
  • parvovirus
  • echovirus
  • HIV
  • human T-cell lymphotropic virus Type 1
  • toxoplasma and Borrelia species

In children, dermatomyositis diagnoses occur more in the months of April and May, suggesting an environmental factor as well. 

Sex and gender exist on spectrums. This article will use the terms “female” and “women” when discussing people assigned female at birth to reflect language that appears in source materials.

Learn more about the difference between sex and gender here.

Autoimmune illnesses 

If you have another autoimmune disorder, you may be more at risk for developing dermatomyositis. Some common autoimmune disorders that may lead to dermatomyositis include:

Underlying cancer 

In 10%-50% of adults with dermatomyositis, an underlying cancer can link to it. Common cancers associated with the disorder are:

Malignancies are more common in adults over the age of 40 to 50. 


Doctors do think that there is a genetic component to dermatomyositis. For instance, although the exact link is not clear, researchers have found that some people with dermatomyositis have a higher number of human leukocyte antigens, which are proteins involved in the immune system. 

If you have other family members with the disorder or other autoimmune disorders, you may have a higher risk of developing dermatomyositis. 

Usually, the disorder appears as a result of both a genetic predisposition and an environmental trigger. 

Read more about the immune system here.

How is heliotrope rash diagnosed? 

If you have symptoms of a rash on your eyelids, you should visit your doctor, who can perform a visual assessment and evaluate your health history and current symptoms. 

Next, doctors can take a skin biopsy of the affected area and order additional tests to confirm or rule out dermatomyositis. To confirm a diagnosis, doctors will usually order:

  • bloodwork to test creatine kinase and aldolase, which check for muscle deterioration and damage
  • bloodwork to test for autoantibodies, which are a sign of an autoimmune condition
  • an MRI to check for muscle inflammation
  • electromyography (EMG) testing to look for decreased electrical activity in the muscles
  • muscle biopsy to confirm a muscle weakness disorder 

How is heliotrope rash treated? 

Usually, the heliotrope rash will clear on its own and fade. Sometimes, it may discolor or change the skin permanently. 

To treat a heliotrope rash, doctors will usually treat the underlying dermatomyositis disorder. 

Dermatomysotisis treatment includes a combination of different therapies, such as:

  • immunosuppressant medications to decrease the immune response, with common medications such as methotrexate, azathioprine, cyclophosphamide, and cyclosporin
  • Intravenous (IV) immunoglobulin
  • steroids to reduce swelling, redness, and inflammation
  • physical therapy and targeted exercises to help maintain muscle strength and ability
  • sun and skin protection to avoid making skin flare-ups worse 

Is dermatomyositis fatal? 

There is no cure for dermatomyositis and the disease affects everyone differently. Many people with dermatomyositis can manage their symptoms with a combination of medications, lifestyle changes, and working closely with their doctors. 

Some people also have symptoms of the disease that come and go. They may have times when symptoms of the disease are very mild and other times when they are worse. 

In very rare cases, if the disease affects the lung and there are other severe symptoms present, the condition may be fatal. If the condition is due to underlying cancer, the risk of life threatening complications may increase. 

Who is at risk for a heliotrope rash? 

According to the National Health Service (NHS), dermatomyositis is more common in women and children. Female adults have the disease twice as often as males.

Anyone with autoimmune disorders, especially those that affect the skin, such as lupus, may also have an increased risk. People with certain types of cancer, like ovarian, breast, and lung cancer, may also be more at risk.

What is the outlook for those with a heliotrope rash?

If you develop a heliotrope rash and receive a diagnosis of dermatomyositis, you should know that the outlook for the condition is very individual. Some people will have mild symptoms of the disease, while others will have more severe symptoms. 

Symptoms of the disease can range from affecting the skin only, to leading to widespread muscle weakness. Dermatomyositis can also affect the joints and internal organs, such as the heart. 

For most people, symptoms will come and go. There may be times when symptoms are worse and times when symptoms improve or disappear completely. However, there is no cure for dermatomyositis and symptoms do tend to get worse over time. 

Many people can manage their symptoms with medications — such as steroids and anti-inflammatory drugs — and exercises that can help. The right exercises can be important for people with any type of progressive muscle weakness disorder. 


A heliotrope rash is a purple-reddish rash that appears on the upper eyelids. The upper eyelids may also appear swollen along with the rash. The heliotrope rash is typically the first sign of a rare progressive inflammatory muscle disease called dermatomyositis. 

Dermatomyositis leads to skin and muscle changes and is more common in adults, with females having the disease twice as often as males. Doctors consider dermatomyositis to be an autoimmune condition, which means that the immune system attacks the body’s own healthy cells. In this case, the immune system is affecting the skin and muscles. 

There is no cure for dermatomyositis, but people with the disorder can typically manage their symptoms through a combination of anti-inflammatory medications, immunosuppressants, and physical therapy. Usually, the heliotrope rash as part of the disease will resolve on its own and fade away. In some cases, the rash may cause permanent skin or color changes. 

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Medical Reviewer: Meredith Goodwin, MD, FAAFP
Last Review Date: 2022 Sep 28
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