Dermatomyosystis

Diagnosis of and treatment for this rare skin condition.

Dermatomyositis is a disease that causes muscle weakness and skin rash. Researchers are unsure what causes this condition. We do know:

• Children 5 to 14 years old and in adults over age 40 most often get this disease
• Past age 55, the disease may be associated with cancer of the lung, stomach, colon, breast, ovary, or uterus

Symptoms of dermatomyosystis

People with this condition usually have a red or violet-colored skin rash on the eyelids, face, chest, knuckles, or around the fingernails. The affected areas are more sensitive to sun exposure.

The disease also causes progressive muscle weakness, often in the hips, thighs, shoulders, and upper arms. This can lead to difficulty climbing steps or rising out of chairs. Patients also may experience:

• Fatigue
• Gastrointestinal ulcers and infections
• Lung problems
• Trouble swallowing

How we diagnose dermatomyosystis

First we perform a physical exam of the affected areas. Then we take a small sample of skin or muscle and analyze it in our lab. This helps us confirm the diagnosis and rule out other diseases or infections.

Your doctor also may recommend a few other exams to check for deeper muscle or tissue damage. These tests include:

• Blood test
• Chest x-ray
• Magnetic resonance imaging (MRI)

Treatment for dermatomyosystis

There currently is no cure for dermatomyosistis, but treatment can greatly improve the skin and muscle symptoms. Treatment options include:

• Antimalarial medications (Plaquenil) for a persistent rash
• High-dose intravenous immunoglobulin therapy
• Immunosuppressant medications, such as methotrexate, azathioprine, or cyclophosphamide
• Man-made antibodies designed to target and destroy specific immune cells. We currently use infliximab (Remicade), rituximab (Rituxan), and etanercept (Enbrel).
• Physical therapy
• Prednisone (corticosteroids)