From the Guidelines
Dermatomyositis is a rare autoimmune disease that causes inflammation of the skin and muscles, and its treatment should prioritize high-dose corticosteroids concurrent with a steroid-sparing agent, such as methotrexate, azathioprine, or mycophenolate mofetil, to manage symptoms and improve quality of life. The condition occurs when the immune system mistakenly attacks the body's own tissues, particularly affecting the skin and muscles, leading to a distinctive rash and muscle weakness that can cause difficulty with everyday activities like climbing stairs or lifting objects above the head 1. Some key symptoms of dermatomyositis include:
- A distinctive rash that appears on the face, eyelids, knuckles, elbows, knees, chest, and back
- Muscle weakness that typically affects the muscles closest to the trunk, such as those in the hips, thighs, shoulders, and neck
- Fatigue, fever, weight loss, and difficulty swallowing Treatment for dermatomyositis typically involves medications that suppress the immune system, and the initial drug regimen should include high-dose corticosteroids concurrent with a steroid-sparing agent, such as methotrexate, azathioprine, or mycophenolate mofetil, followed by a tapering course of corticosteroids 1. Additionally, physical therapy is important to maintain muscle strength and function, and novel biomarkers of disease activity such as interleukin 6 and type 1 interferon-regulated genes may serve as indicators of disease activity in adult and juvenile myositis 1. While there's no cure for dermatomyositis, treatment can help manage symptoms and improve quality of life, and scientists believe that genetic factors combined with environmental triggers like viral infections or certain medications may play a role in its development.
From the Research
What is Dermatomyositis
- Dermatomyositis (DM) is an autoimmune disorder characterized by skin and muscle involvement 2, 3, 4.
- It belongs to a group of rare autoimmune dermatoses with different skin features and variable muscle involvement 2.
- The condition can be categorized into four main variants: classic DM, clinically amyopathic DM, paraneoplastic DM, and juvenile DM 2.
Symptoms of Dermatomyositis
- Patients with DM show several skin features, including heliotrope rash and violaceous papules located at the interphalangeal or metacarpophalangeal joints (Gottron's papules) 2.
- Muscle involvement is also common, with symmetrical weakness of the proximal muscles 2.
- DM can also be associated with various systemic manifestations, including pulmonary disease or an associated malignancy 4.
Treatment of Dermatomyositis
- Systemic corticosteroids are considered the first-line approach for treating DM 2, 5.
- Steroid-sparing agents, such as methotrexate, azathioprine, or mycophenolate mofetil, can be effective in treating DM 2, 6, 5.
- New classes of drugs, including monoclonal antibodies, purified immunoglobulins, or Janus kinase inhibitors, are becoming more relevant in clinical practice or are currently under investigation 2, 6.