Heliotrope Rash

Heliotrope rash is a rare inflammatory condition that is characterized by weakness of the muscles and a marked rash on the skin. It is medically categorized as a persistent inflammatory myopathy and only one of 3 such disorders. Heliotrope rash gets its name from the tendency of plants to develop towards sunlight, i.e. heliotropic. The condition is also often called dermatomyositis.

Heliotrope rash can occur in children as well as adults. Adults in the age group of late forties and early sixties and children in the age group of five and fifteen years are most vulnerable to the condition. The disease is also more prevalent in females as compared to males

Patients may experience remission period when the symptoms of heliotrope rash may suddenly improve. Treatment can help in regaining muscle functionality and power, and also in clearing the rash.

Symptoms of heliotrope rash

Some of the signs and symptoms of heliotrope rash are as follows:

• Patients of heliotrope rash may experience a dusky reddish or violet colored skin anomaly, often on the eyelids and the face, as well as around the knuckles, nails, knees, elbows, back and chest region. The rash has a tendency to be blotchy with blue-purple stains and is mostly the first symptom of the condition.
• The muscles occurring in the areas next to the torso such as the shoulders, thighs, neck, hips and upper arms may elicit progressive weakness of the muscles. Such weakness has a symmetric pattern and may affect both the right and left sides of the body, with a tendency to slowly exacerbate.
• Swallowing difficulties may be observed
• Loss of weight, exhaustion and fever may be evident
• Tenderness and pain of the muscles
• Accumulation of thickened calcium beneath the skin may occur, more so in children
• Disorders of the lungs
• Intestinal punctures and gastrointestinal ulceration may also develop, especially in affected children

Some of the complications associated with heliotrope rash are discussed below:

• Complications of muscle weakness:
  • When the esophagus experiences muscle weakness then patients may elicit dysphagia or swallowing difficulties. This in turn can result in loss of weight and malnutrition
  • Cases of heliotrope rash which lead to chest muscle weakness may cause breathing disturbances, such as breathlessness
  • Patients may also develop aspiration pneumonia due to swallowing problems. Patients may aspirate fluids, food, or saliva into the lungs which causes the complication
  • Ulcer formation, bleeding and other gastrointestinal problems may also be noticed
  • Complications of skin symptoms
    • Cases of heliotrope rash pose elevated risk to infections, especially of the digestive and respiratory tracts
    • Deposition of calcium in the skin, muscles and connective tissues may also form with the advancement of the disorder. Such accumulation is more common and occurs earlier in children with heliotrope rash
    • Accompanying diseases
      • Heliotrope rash increases the risk of patients to Raynaud’s phenomenon which features discoloration of the toes, fingers, nose, cheeks, ears and other extremities during times of exposure to cold weather
      • Lupus, scleroderma, rheumatoid arthritis, Sjogren’s syndrome and conditions of the connective tissue can also develop along with heliotrope rash
      • Interstitial lung disease which is a cluster of diseases marked by lung tissue scarring or fibrosis, which results in inelasticity or stiffness of the lungs, may also occur along with heliotrope rash. The disease can cause breathlessness and a dry cough
      • Heliotrope rash can also result in inflammation of the cardiac muscles or myocarditis, and other cardiovascular conditions. A small percentage of the patients may also experience cardiac arrhythmias and congestive failure of the heart.
      • Adults affected by heliotrope rash are at increased vulnerability to cancers, especially of the lungs, cervix, ovaries, breasts and the gastrointestinal tract.
      • An underlying case of heliotrope rash can result in aggravated symptoms in pregnant women. They are greater susceptibility to suffering from stillbirth or premature delivery of the child. The risk to such complications diminish if heliotrope rash is in remission

Causes

The precise cause of heliotrope rash is not known. However, the disease is somewhat similar to autoimmune conditions which cause the immune system to attack healthy body tissues.

It is known that immune system is responsible for protecting the body against attack by infections, bacteria and other pathogens. People with polymyositis, may elicit an abnormal response from the immune system, due to unknown reasons, which result in formation of antibodies that fight the normal tissues of the body.

The tiny blood vessels occurring in the muscular tissues are often affected by heliotrope rash. The inflammatory cells enclose the blood vessels and ultimately result in deterioration of the muscle fibers

Treatment of heliotrope rash

• There is no cure for heliotrope rash. Treatment is aimed at managing and improving the symptoms
• Corticosteroid medications may be prescribed by doctors to suppress the immune system. In case, heliotrope rash does not react positive to corticosteroid medications, then other immunosuppressive therapies, such as intravenous immunoglobulin, etc. may be tried.
• Biological therapies which include Rituximab or TNF inhibitors are other ways to treat the symptoms of heliotrope rash
• Pain can be alleviated with pain killer drugs
• Physical therapy can help in regaining muscle strength and flexibility.

Heliotrope rash pictures