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Dermatomyositis – Causes, Symptoms and Treatment

Dermatomyositis is an inflammatory disease affecting both the skin and the muscles. It belongs to the group of idiopathic inflammatory myopathies and requires early diagnosis and treatment.

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Things worth knowing about "Dermatomyositis"

Dermatomyositis is an inflammatory disease affecting both the skin and the muscles. It belongs to the group of idiopathic inflammatory myopathies and requires early diagnosis and treatment.

What is Dermatomyositis?

Dermatomyositis is a rare, chronic inflammatory disease that simultaneously affects the skin and the skeletal muscles. It belongs to the group of idiopathic inflammatory myopathies (IIM) and is characterized by distinctive skin changes and progressive muscle weakness. The condition can occur at any age but more commonly affects women than men. A special form, known as juvenile dermatomyositis, occurs in children and adolescents.

Causes and Risk Factors

The exact cause of dermatomyositis has not yet been fully established. It is believed that a combination of genetic predisposition, environmental factors, and a dysregulation of the immune system contributes to the disease. The immune system mistakenly attacks the body´s own tissue in the skin and muscles (autoimmune reaction).

Genetic factors (specific HLA genes)
Viral or bacterial infections as possible triggers
UV radiation (can worsen skin lesions)
Association with malignant tumors (paraneoplastic dermatomyositis), particularly in adults
Certain medications (drug-induced myositis)

Symptoms

Dermatomyositis presents a wide range of symptoms that can affect the skin, muscles, and internal organs.

Skin Changes

Gottron papules: Reddish to violet, scaly skin elevations over the finger joints -- a hallmark sign of the disease
Heliotrope rash: Violet discoloration of the eyelids, often accompanied by swelling
Shawl sign: Rash affecting the shoulders, neck, and upper back
Mechanic hands: Cracked, thickened skin on the palms and fingers
Nail fold changes (dilated capillaries)

Muscle Symptoms

Symmetrical, proximal muscle weakness (shoulder and pelvic girdle)
Difficulty rising from a chair, climbing stairs, or lifting the arms
Muscle pain and tenderness
In severe cases: swallowing difficulties (dysphagia) due to involvement of the pharyngeal muscles

General Symptoms

Fatigue and general malaise
Weight loss
Fever
Rarely: lung involvement (interstitial lung disease) or inflammation of the heart muscle

Diagnosis

The diagnosis of dermatomyositis is based on clinical findings, laboratory tests, and imaging procedures.

Blood tests: Elevated muscle enzymes (creatine kinase, LDH, aldolase), detection of specific autoantibodies (e.g., anti-Jo-1, anti-Mi-2, anti-MDA5)
Electromyography (EMG): Detection of myopathic changes in the muscles
Muscle MRI: Visualization of inflammatory lesions within the muscle tissue
Muscle biopsy: Histological examination of muscle tissue -- considered the gold standard for diagnosis
Skin biopsy: Used when skin changes are unclear
Cancer screening: In adults, a comprehensive malignancy workup should be performed

Treatment

Treatment of dermatomyositis is individualized and depends on the severity of the disease and the presence of associated conditions.

Medical Therapy

Corticosteroids (e.g., prednisone): First-line treatment for rapid reduction of inflammation
Immunosuppressants: Methotrexate, azathioprine, or mycophenolate mofetil for long-term therapy and steroid sparing
Intravenous immunoglobulins (IVIG): Used in severe or treatment-resistant cases
Rituximab: A biologic agent for refractory disease
Hydroxychloroquine: Supportive treatment for skin manifestations

Non-Medical Therapy

Physiotherapy to maintain and improve muscle strength
Sun protection to shield the skin from UV radiation
Speech therapy in cases of swallowing difficulties

Prognosis and Disease Course

The course of dermatomyositis is variable. Some patients achieve lasting remission with treatment, while others experience a chronic, relapsing course. When associated with a malignancy, the myositis often improves following successful cancer treatment. Regular follow-up examinations are essential to detect relapses at an early stage.

References

Bohan A, Peter JB. Polymyositis and dermatomyositis. New England Journal of Medicine. 1975;292(7):344-347.
Lundberg IE et al. 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies. Annals of the Rheumatic Diseases. 2017;76(12):1955-1964.
Dalakas MC. Inflammatory muscle diseases. New England Journal of Medicine. 2015;372(18):1734-1747.

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