Heliotrope Rash - Causes, Symptoms, and Management

What Is Heliotrope Rash?

Heliotrope rashes are caused by a rare connective tissue disease called dermatomyositis. It is a reddish-purple rash that commonly appears on the upper eyelids and other body areas. It is often associated with swelling at the same site. The patient may also see other symptoms, such as fever, joint pain, or weakness. Moreover, the rash presents as an itch or burning sensation on the touch, which worsens with sun exposure. Only 10 cases per million adults are diagnosed with this condition. Females are affected more than men.

What Are the Common Sites Where the Heliotrope Rash Appears?

These are the following sites of heliotrope rash that appear:

• Face.

• Neck.

• Knuckles.

• Upper eyelids.

• Elbows.

• Chest.

• Back.

• Knees.

• Shoulders.

• Hips.

• Nails.

What Are the Causes of Heliotrope Rash?

Heliotrope rashes are symptoms associated with dermatomyositis. The following are the etiological factors associated with a heliotrope rash:

1. Genetics: The condition is believed to carry genetic inheritance, which means the condition runs in the family.

2. Autoimmune Disease: The body's immune system has an important function, fighting bacteria or infections that invade our body. If a person has a compromised immune system, there are chances that this mechanism will not work efficiently. This can lead to the body's healthy cells getting attacked by the immune system. A rash is a complication among many others with similar unexplained etiology. The following are the autoimmune disorders associated with dermatomyositis:

• Lupus: It is an inflammatory condition brought on when the body's immune system attacks its cells, which causes complications related to the brain, joints, skin, kidneys, heart, lungs, and blood cells. The most common sign of this condition is a butterfly shaped rash covering the cheeks of the face. These rashes can either be a result of genetic or the triggers caused by infection, drug side effects, or sunlight.

• Rheumatoid Arthritis: It is a chronic inflammatory disease affecting the joints of a person's hands and feet, making it a painful condition. The condition is caused by the immune system attacking the body's healthy cells. However, the actual reason behind this disturbed autoimmune system is still unknown.

• Scleroderma: It is systemic sclerosis affecting the skin, blood vessels, digestive tract, and internal organs of the body. The condition is often characterized by limited or diffused types, which are either vascular or cause organ problems. There is no cure for the condition, only the symptoms can be managed.

• Sjogren's Syndrome: It is an autoimmune disorder causing dry eyes and mouth. This disorder affects the body's cells, which help produce tears and saliva, and attacks them. The etiology of this disorder is genetic or environmental. Medications such as Hydroxychloroquine can help with the symptoms.

• Mixed Connective Tissue Disease: It is a condition with combined signs and symptoms of disorders. These disorders are lupus, scleroderma, and polymyositis. It is a complicated condition, as it takes years to show the symptoms.

3. Cancer: If a person has dermatomyositis, the chances of developing cancer increase. Due to this, many types of research are going on to check the relationship between cancer genes and this disorder.

4. Infection: In the development of dermatomyositis, the exposure to toxins and the triggers that cause this condition play an essential role. The following triggers increase the chances of developing dermatomyositis, hence the heliotrope rash:

• Coxsackie virus.

• Parvovirus.

• Echovirus.

• Human immunodeficiency virus (HIV).

• Human T-cell lymphotropic virus type-I.

• Toxoplasma and borrelia species.

5. Adverse Effect of Medication: Adverse effects related to some medications can also lead to this complication.

What Are the Signs and Symptoms of Heliotrope Rash?

Other than a purple-reddish patch and swelling at the site, other symptoms are associated with this. The followings signs and symptoms are associated with a heliotrope rash:

• Ragged cuticles along with exposed blood vessels.

• Scaly scalp.

• Skin swelling.

• Weakness.

• Yellow-white bumps under the skin.

• Thin hair.

• Pale, thin, and sensitive skin.

Who Is at the Risk of Developing Heliotrope Rash Along With Dermatomyositis?

Having dermatomyositis increases the chances of developing heliotrope rashes on the skin. The following factors increase the chances of the development of this condition:

Cancer: Dermatomyositis increases the chance of developing cancer in anyone by three to eight percent.

Tissue Disease: Dermatomyositis and heliotrope rashes are part of connective tissue disorders. Due to this reason, the chances of developing tissue disease also increase when having this condition.

Lung Disorders: Heliotrope rashes and dermatomyositis affect lung health. People with this condition develop complications such as shortness of breath and coughing. The study shows that almost 35 to 40 percent of people have increased chances of developing interstitial lung disease with this condition.

How to Diagnose Heliotrope Rash?

There are the following diagnostic methods used for a heliotrope rash:

Physical Examination: Heliotrope rashes have very specific features, such as reddish-purple rashes over the upper eyelids. The doctor may evaluate the symptoms with this.

Blood Analysis: Increased levels of enzymes and antibody markers are suggestive of the condition. After a physical examination, doctors may further use diagnostic methods to confirm the condition.

Biopsy: Tissue biopsy is the next step after blood analysis. Taking a sample of muscle or affected skin with a rash can further confirm the condition.

Imaging: Magnetic resonance imaging or X-ray can confirm any complications occurring in the body. Moreover, this diagnostic step can also help rule out underlying causes.

Cancer Screening: The condition increases the chances of having cancer. Hence, doctors suggest full-body screening to test for cancer.

How to Manage Heliotrope Rash?

Management options for this condition depend on the severity of the condition and the time taken for the diagnosis. The following management options are available for a heliotrope rash:

• Antimalarials: This medication helps with the rashes caused by dermatomyositis.

• Sunscreen: If the rashes are aggravated due to prolonged sun exposure, sunscreen is suggested. Sunscreen provides a protective layer on the skin.

• Oral corticosteroids: Corticosteroids such as Prednisone helps to relieve the symptoms such as rashes.

• Immunosuppressants: The condition is caused by a defective immune system. Medications such as Methotrexate and Mycophenolate help.

Conclusion

Heliotrope rashes are one complication of dermatomyositis among many complications caused by this condition. Due to its complicated symptoms and underlying triggers, it is difficult to diagnose. Therefore, a person needs to seek medical attention on the appearance of the first symptom.