adult dermatomyositis

Adult Dermatomyositis: A Rare Autoimmune Disease

Dermatomyositis (DM) is a rare autoimmune disease that affects adults, characterized by muscle inflammation and skin rash. The condition is part of a group of diseases called inflammatory myopathies, which involve chronic muscle inflammation, muscle weakness, and in some cases, muscle pain.

Key Features:

• Muscle Inflammation: DM causes inflammation of the muscles, particularly those closest to the trunk (proximal muscles), such as the upper arms and thighs.
• Skin Rash: A distinctive red or purplish rash appears on the skin, often in areas where muscles are used to straighten joints, like the knuckles, elbows, and knees.
• Muscle Weakness: Muscle weakness is a common symptom of DM, affecting the proximal muscles.
• Increased Cancer Risk: People with DM have an increased risk of developing certain types of cancer, including ovarian, lung, lymphoma, breast, and colon cancer.

Other Complications:

• Heart Muscle Inflammation: In some cases, DM can cause heart muscle inflammation, leading to congestive heart failure and heart rhythm problems.
• Lung Disease: Interstitial lung disease can occur with DM, causing scarring of lung tissue and making it difficult to breathe.

Diagnosis:

Diagnosis of DM is based on a combination of clinical features, laboratory tests, and imaging studies. These include:

• Muscle enzymes (e.g., creatine kinase) are elevated.
• Electromyography shows myopathic changes.
• Muscle biopsy reveals characteristic muscle damage.
• Skin rash and other dermatological manifestations.

Treatment:

While there is no cure for DM, treatment options are available to manage symptoms and slow disease progression. These include corticosteroids, immunosuppressive medications, and physical therapy to improve muscle strength and function.

References:

[1] Dermatomyositis has also been found to increase your risk of developing certain types of cancer (Source: [2]) [2] Some of the most common cancers people with dermatomyositis develop include: Ovarian cancer, Lung cancer, Lymphoma, Breast cancer, Colon cancer. (Source: [3]) [3] Dermatomyositis is a rare disease that causes muscle inflammation and skin rash. It’s one of a group of muscle diseases that cause muscle inflammation and swelling. (Source: [4]) [4] Dermatomyositis is a disease that involves muscle inflammation and a skin rash. Polymyositis is a similar inflammatory condition that also involves muscle weakness, swelling, tenderness, and tissue damage but no skin rash. (Source: [5])

Common Signs and Symptoms of Adult Dermatomyositis

Adult dermatomyositis, a rare autoimmune disease, can manifest in various ways. The most common signs and symptoms include:

• Skin manifestations: A distinctive reddish-purple rash (heliotrope rash) on the upper eyelid or across the cheeks [8]. A patchy, red rash, usually on the eyelids, face, neck, and the backs of the hands [9].
• Muscle weakness: Progressive muscle weakness, which can affect any muscle group, including the torso or "core" [12]. Difficulty rising from a low-seated chair or combing one's hair [12].
• Lung issues: Lung inflammation that causes extreme shortness of breath [10].
• Other symptoms: Painful or aching muscles, feeling very tired, weight loss, night sweats, and fever [9].

Important Notes

• Dermatomyositis can develop quickly or gradually over time [2].
• The sooner treatment begins, the more likely it is to avoid severe complications [2].
• Women are at least two times more likely to suffer from dermatomyositis than men [11].

Treatment Options for Adult Dermatomyositis

Adult dermatomyositis, a rare autoimmune disease characterized by muscle weakness and skin rash, requires prompt and effective treatment to manage symptoms and prevent long-term damage.

Medications Used in Treatment

Several medications are commonly used to treat adult dermatomyositis. These include:

• Corticosteroids: Prednisone is often the first-line therapy for muscle involvement in dermatomyositis [5]. The dose is altered according to the response of the patient's condition.
• Immunosuppressive/immunomodulatory agents: In addition to corticosteroids, other medications such as azathioprine (Azasan, Imuran), methotrexate (Trexall), and mycophenolate mofetil (Cellcept) may be prescribed [1].
• Glucocorticoids: Glucocorticoids, particularly prednisone, are often the first-line treatment for dermatomyositis. These medications help to reduce inflammation and swelling, and suppress damaging immune responses [7].

Newer Treatment Options

Recent studies have explored newer treatment options for adult dermatomyositis, including:

• JAK inhibitors: A pilot study has investigated the use of JAK inhibitors in treating adult dermatomyositis, with promising results [8].
• Intravenous immunoglobulin (IVIg): IVIg has been shown to be an effective short-term treatment for refractory dermatomyositis, although its long-term effects remain unknown [9].

Treatment Guidelines

The American Academy of Dermatology recommends a stepwise approach to treating adult dermatomyositis, starting with glucocorticoids and adding immunosuppressive/immunomodulatory agents as needed. A recent review outlines the selection of agents for treatment [6].

References:

[1] Context 1 [5] Context 5 [7] Context 7 [8] Context 8 [9] Context 9

Differential Diagnosis of Adult Dermatomyositis

Adult dermatomyositis (DM) is a rare autoimmune disease characterized by skin and muscle inflammation. When diagnosing DM, it's essential to consider other conditions that may present with similar symptoms. Here are some differential diagnoses for adult DM:

• Polymyositis: This is another idiopathic inflammatory myopathy that can cause muscle weakness and pain, often without skin manifestations [3].
• Inclusion Body Myositis (IBM): A rare and progressive muscle disease characterized by muscle weakness and wasting, which may be confused with DM due to similar symptoms [3].
• Calcium Deposition (Calcinosis Cutis): This condition can occur in approximately 30-70% of juvenile DM cases but is less common in adults. However, it's essential to consider calcinosis cutis as a differential diagnosis for adult DM patients with skin manifestations [4].
• Lupus Erythematosus: A chronic autoimmune disease that can cause skin and joint inflammation, which may be confused with DM due to similar symptoms [2].
• Graft Versus Host Disease (GVHD): A condition that occurs when the immune system of a transplant recipient attacks their own body. GVHD can cause skin and muscle inflammation, making it a differential diagnosis for adult DM [2].

Key Points

• Polymyositis and IBM are other idiopathic inflammatory myopathies that should be considered in the differential diagnosis of adult DM.
• Calcinosis cutis is less common in adults but essential to consider when diagnosing DM patients with skin manifestations.
• Lupus erythematosus and GVHD can cause similar symptoms to DM, making them important differential diagnoses.

References

[1] Christopher-Stine L. Dermatomyositis: A Review of the Literature. [online] Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7181119/ [Accessed 6 Dec. 2024].

[2] Jun 29, 2023 — Discoid Lupus Erythematosus · Graft Versus Host Disease (GVHD) · Lichen Myxedematosus · Lichen Planus · Multicentric Reticulohistiocytosis.

[3] What is the differential diagnosis for adult-onset dermatomyositis? · Other idiopathic inflammatory myopathies, such as polymyositis or inclusion body myositis ...

[4] by RICA KOLER · 2001 · Cited by 138 — Differential Diagnosis of Dermatomyositis​​ Calcium deposition (calcinosis cutis) occurs in approximately 30 to 70 percent of cases of juvenile ...