What is Heliotrope Rash: Causes, Symptoms, Treatment, Signs, Diagnosis

What is Heliotrope Rash?

Heliotrope rash is the primary symptom of Dermatomyositis, which is an idiopathic inflammatory myositis. Patients suffering from dermatomyositis have persistent inflammation of the skin and muscle. There is development of heliotrope rash on the upper eyelids along with inflammation. Heliotrope rash is often associated with violaceous erythema of the upper eyelids along with telangiectasia and edema which progresses into heliotrope rash. ‘Heliotrope’ is a term which is derived from the heliotrope flower, because in this rash, the color of the skin turns to violet, which is the color of the heliotrope flower.

What is Heliotrope Rash?

Associated Signs & Symptoms of Heliotrope Rash

Heliotrope rash occurs during the initial phase of the idiopathic inflammatory myositis, particularly dermatomyositis. Violaceous discoloration and periorbital edema is the primary feature of Heliotrope rash. Patient experiences itching of the skin along with ragged cuticles. There is also development of muscle weakness with Heliotrope rash.

Additional symptoms of Heliotrope rash include:

Shawl Sign: The main feature of shawl sign is flat, reddish-purple discoloration, which extends over a wide area of the skin including shoulders, neck, arm etc. Exposure to the sun (UV rays) worsens the condition of Heliotrope rash.

Gottron’s Sign: This develops on the elbow, knees and fingers. Patient has a red and flat rash on the posterior side of the knees, fingers and elbows.

Gottron’s Papules: This is similar to Gottron’s sign, but instead of a rash which is flat, there is development of scaly bumps on the joints of the fingers.

V-Sign: This is similar to shawl sign; however, it develops on the chest with the rash shape resembling a v-necked sweater.

Mechanic’s Hands: The patient’s hands appear dirty, due to skin of the hand becoming cracked, along with brittle and rough nails which have irregular tips and irregular side of the nails. This makes the appearance of the hands like a manual mechanics’ hands.

Reddish Nail Bed: The nail bed of the finger becomes reddish from inflammation of the blood vessels surrounding it.

Causes of Heliotrope Rash

Dermatomyositis (DM) is a clinical condition where there is appearance of rash on the skin with inflammation of the skeletal muscle.

Juvenile Dermatomyositis (JDM) is similar to dermatomyositis, but is found in children, as the name itself suggests.

Amyopathic Dermatomyositis (ADM) is a condition, which involves only cutaneous tissues. The muscles are not affected and the patient has different types of rashes.

Some of the potential causes of the above mentioned conditions are: Skin malignancy, genetic abnormality, exposure to natural infectious agents/toxic chemicals, side effect of certain drugs, such as quinidine, penicillamine, hydroxyurea, phenylbutazone and statin.

Diagnosis of Heliotrope Rash

Given below are the investigations, which are done to diagnose and confirm the underlying cause of Heliotrope Rash:

  • Biopsy of the skin where a sample of the skin from the affected area is taken and sent to lab for testing.
  • Serologic tests are done to help identify the cause of heliotrope rash. Serologic testing done for myositis-specific antibodies is helpful.
  • Enzymatic testing comprising of estimation of creatine kinase, alanine aminotransferase, lactic dehydrogenase or aldolase is done. The increased level of lactic dehydrogenase and creatine kinase helps in diagnosis.
  • Von Willebrand factor detection is done for diagnosing Juvenile Dermatomyositis (JDM).

The below tests are done to assess the severity of the condition and include:

  • Chest x-ray and pulmonary function test is done to check if there is lung involvement.
  • Swallowing evaluation and esophageal motility studies are done for assessing the esophagus involvement.
  • Electrocardiogram is done to assess the cardiac function.

Treatment of Heliotrope Rash

Treatment depends on the diagnosis of the underlying cause of Heliotrope Rash and consists of:

  • Rest along with adequate stretching along with splinting physical manipulation to help alleviate the muscle weakness associated with heliotrope rash.
  • Patient should avoid direct sunlight and should use a sunscreen with high SPF and ultraviolet ray protection. Patient should also wear protective clothing and other accessories when stepping out in the sun.
  • Anti-histaminic ointment for heliotrope rash is prescribed to relieve the itching and inflammation of the skin.
  • Steroids, such as prednisone and corticosteroids, can also be prescribed to treat heliotrope rash. The dose depends on the weight of the patient. Steroidal therapy often provides complete remission of Heliotrope Rash. If there is recurrence, then steroidal therapy is started.
  • Immunosuppressants, such as cyclophosphamide, methotrexate, azathioprine, mycophenolate mofetil or cyclo sporinare chlorambucil are also prescribed for maintenance and for prevention of recurrence of heliotrope rash.
  • Tacrolimus ointment and pimecrolimus cream also help in alleviating the symptoms of heliotrope rash by acting as immunomodulator.
  • If there is involvement of the heart, then antihypertensive medicines are prescribed accordingly.
Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:January 23, 2020

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