Dermatomyositis: Symptoms, Causes, Diagnosis, and Treatment

Dermatomyositis, a rare autoimmune disease, presents a unique challenge in the realm of rheumatology and dermatology. Characterised by muscle weakness and skin inflammation, it can significantly impact patients’ quality of life.

Understanding its complex aetiology, varied clinical manifestations, and evolving treatment approaches is crucial for healthcare professionals and individuals affected by this condition. In this blog, we delve into the intricacies of dermatomyositis, shedding light on its diagnosis, management, and the latest research advancements.

What is Dermatomyositis?

Dermatomyositis, a rare myopathy, manifests as muscle weakness and skin rashes. Severe cases may impair breathing and swallowing. It’s a variant of polymyositis affecting both muscles and skin.

Prompt medical attention is vital as symptoms can escalate quickly. Early treatment reduces the risk of severe complications and potential fatality, also lowering the chance of cancer development, especially within the first year of symptoms.

What are the Types of Dermatomyositis? 

An uncommon inflammatory condition that affects the muscles and skin is called dermatomyositis. There are several types of dermatomyositis, including:

1. Classic Dermatomyositis:

   This is the most common type and is characterised by muscle weakness and a distinctive skin rash.

2. Amyopathic Dermatomyositis:

   In this type, there is skin involvement without significant muscle weakness. It’s also known as dermatomyositis sine myositis.

3. Homoeopathic Dermatomyositis:

   This type involves mild muscle weakness alongside skin symptoms.

4. Paraneoplastic Dermatomyositis:

   This form of dermatomyositis is associated with an underlying cancer, most commonly lung, ovarian, or gastrointestinal cancers.

5. Childhood Dermatomyositis:

   This type affects children and typically presents with similar symptoms to classic dermatomyositis.

Dermatomyositis types vary in presentation, severity, and treatment. Consultation with healthcare professionals is essential for diagnosis and management.

What are the Symptoms of Dermatomyositis?

Dermatomyositis manifests through various symptoms, primarily muscle weakness and skin rash. These often occur concurrently, although one symptom may precede the other for extended periods.

Muscle weakness affects daily activities like:

• Sitting upright becomes challenging.
• Difficulty standing from a seated position.
• Climbing stairs or rising after lying down becomes strenuous.
• Simple tasks like washing hair become arduous.

The rash, common in sun-exposed areas, appears discoloured and swollen on:

• Eyelids and around the eyes.
• Chest and front shoulders (v-sign rash).
• Neck and back shoulders (shawl sign rash).
• Scalp may also be affected.

Additional symptoms include:

• Gottron papules on hands.
• Calcium deposits.
• Bumps on knees or elbows.
• Ragged cuticles and prominent blood vessels.
• Joint pain.

Dermatomyositis can either resolve, especially in children or persist chronically, causing lifelong symptoms in 80% of cases.

What are the Causes of Dermatomyositis?

Dermatomyositis’s exact cause remains uncertain among experts, although several potential factors are considered:

1. Genetic Influences:

   Research suggests a genetic component to dermatomyositis.

2. Autoimmune Dysfunction:

   Dermatomyositis shares similarities with autoimmune disorders wherein the immune system mistakenly targets healthy tissues.

3. Viral Infections:

   Some evidence indicates that certain viral infections could serve as triggers for dermatomyositis, even post-resolution of the initial infection.

4. Environmental Factors:

   Studies propose a correlation between residing in regions with increased pollution or poorer air quality and heightened susceptibility to dermatomyositis development.

How is Dermatomyositis Diagnosed?

The doctor assesses symptoms, and medical history, and conducts a physical exam. Dermatomyositis, characterised by an autoimmune rash, is comparatively easier to diagnose due to its distinct features among inflammatory muscle diseases.

Your healthcare provider might also request:

• An MRI scan to detect irregularities in muscles.
• Electromyography (EMG) to capture the electrical signals governing muscle activity.
• Blood tests to assess levels of muscle enzymes and autoantibodies, which target healthy cells.
• A muscle biopsy to investigate inflammation and other disease-related issues within a muscle tissue sample.
• A skin biopsy to examine disease-induced changes in a skin tissue sample.

What is the Treatment for Dermatomyositis?

Dermatomyositis treatments may involve a combination of the following:

• Corticosteroids: These medications alleviate muscle inflammation.
• Physical Therapy: Exercise aids in muscle repair and strength enhancement.
• Immunosuppressant Medications: These drugs prevent the immune system from attacking healthy tissues, slowing down muscle damage.
• Intravenous Immunoglobulin (IVIg): Infusions of additional immunoglobulin can complement immunosuppressants or serve as an alternative therapy.
• Speech Therapy: Strengthening throat muscles can aid swallowing if muscle weakness is present.

Your treatment plan depends on symptom location and severity. Consult your healthcare provider to understand the expected course of treatment.

What are the Complications of Dermatomyositis?

The muscle weakness and skin issues associated with dermatomyositis can lead to various complications, including:

1. Development of skin ulcers.
2. Occurrence of gastric ulcers.
3. Challenges in breathing.
4. Susceptibility to lung infections.
5. Difficulty in swallowing.
6. Risk of malnutrition and subsequent weight loss.

Additionally, dermatomyositis may be correlated with the following conditions:

1. Raynaud’s phenomenon.
2. Myocarditis.
3. Interstitial lung disease.
4. Other connective tissue disorders.
5. Elevated likelihood of cancer development.

How Can you Prevent Dermatomyositis?

Preventing dermatomyositis involves a multifaceted approach:

• Healthy lifestyle: Maintain balanced nutrition, regular exercise, and a healthy weight.
• Sun protection: Shield skin from sunlight with sunscreen, clothing, and hats.
• Avoid triggers: Minimise exposure to potential triggers like infections or certain chemicals.
• Stress management: Employ relaxation techniques, meditation, or therapy to manage stress.
• Symptom monitoring: Stay vigilant for signs like muscle weakness or skin rashes and seek prompt medical attention.
• Follow medical advice: Adhere to prescribed medications, attend check-ups, and make lifestyle adjustments as advised.

These strategies, while not guaranteeing prevention, can help mitigate risks and manage symptoms effectively.

Conclusion

Dermatomyositis presents complex challenges, impacting both the skin and muscles. With ongoing research and multidisciplinary care, patients can find hope for improved management and quality of life. It is always advisable to seek medical help from an experienced dermatologist. Timely care and help can ensure an appropriate diagnosis and treatment of your condition. 

At the CK Birla Hospital, we ensure patients get holistic medical support which includes treatment in a compassionate environment. This patient-centric approach not only helps patients heal better but also ensures they are aware of the preventive measures as well. In case you need to consult a dermatologist, reach out to us, or book a direct appointment with dermatologist at the CK Birla Hospital.

FAQs About Dermatomyositis

Can Dermatomyositis Affect Children?

Yes, dermatomyositis can affect children, although it’s less common compared to adults. It’s a rare autoimmune disease characterised by muscle weakness and skin rash. Early diagnosis and treatment are crucial.

Is Dermatomyositis Contagious?

No, dermatomyositis is not contagious. It’s an autoimmune disease where the body’s immune system mistakenly attacks its tissues, leading to muscle weakness and skin rash.

Can Dermatomyositis Lead to Disability?

Yes, dermatomyositis can lead to disability, particularly if not properly managed. Muscle weakness and inflammation can affect mobility and daily activities, impacting quality of life.

Are There Specific Triggers that Worsen Dermatomyositis Symptoms?

Yes, triggers such as sunlight exposure, infections, certain medications, and stress can exacerbate dermatomyositis symptoms, leading to increased muscle weakness and skin rash.

What Lifestyle Changes Can Help Manage Dermatomyositis Symptoms?

Managing dermatomyositis involves regular exercise to maintain muscle strength, sun protection to minimise skin rash, a balanced diet for overall health, stress management, and avoiding triggers like certain medications.