Dermatomyositis

What Is Dermatomyositis?

Dermatomyositis is a rare autoimmune systemic disease of the connective tissue that is characterized by inflammatory and debilitating degenerative changes in the muscles and in the skin. 

Dermatomyositis results in symmetric, proximal muscle weakness of limbs (upper arms and legs), and skin manifestations. 50-70% of patients have circulating myositis-specific auto-antibodies.

The course of dermatomyositis is unpredictable being marked by spontaneous flare-ups and remissions. It can begin slowly or abruptly according to the factor that is triggering the onset such as infection, medications like phenytoin, and autoimmune disease.

Q: What are the skin manifestations of dermatomyositis?

A: Classic skin manifestations of dermatomyositis include these features:

• The heliotrope rash (lilac color) on upper eyelids.
• Rash on face, neck, shoulders, upper chest, elbows, knees, knuckles, and back.
• Gottron’s papules (scaly, red eruptions or  patches over the knuckles, elbows, and knees).
• The V-sign (rash front of neck and chest).
• The shawl sign (rash distribution on shoulders and back).1

Additional cutaneous manifestations are described below under symptoms.

Dermatomyositis is associated with an increased risk of  cancer, other autoimmune diseases, such as lupus and psoriasis, and it can be a complication of interferon-α therapy. About 1 person in 100,000 are affected according to various studies. While it affects all ages, women have twice the occurence of men.

There is no cure for dermatomyositis, but the symptoms can be treated.  Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest.  The standard treatment for dermatomyositis is a corticosteroid drug, given either in pill form or intravenously.  Immunosuppressant drugs, such as azathioprine and methotrexate, may reduce inflammation in people who do not respond well to prednisone.2

What Is Dermatomyositis In Celiac Disease and/or Gluten Sensitivity?

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1. Marvi U, Chung L, Fiorentino DF. Clinical presentation and evaluation of dermatomyositis. Indian J Dermatol. 2012 Sep;57(5):375-81. doi: 10.4103/0019-5154.100486. [↩]
2. National Institute of Neurological Disorders and Stroke. [↩]