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Dermatomyositis Updated 4/2010

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BASICS

  • Description
  • Epidemiology
  • Risk Factors
  • Pathophysiology
  • Etiology
  • Associated Conditions

DIAGNOSIS

  • Signs and Symptoms
  • Tests
  • Differential Diagnosis

TREATMENT

  • Initial Stabilization
  • General Measures
  • Special Therapy
  • Medication (Drugs)

Follow-Up

  • Disposition
  • Prognosis
  • Complications
  • Patient Monitoring
The following is an excerpt....
BASICS
Description
  • Dermatomyositis is an idiopathic inflammatory myopathy which involves the muscles and skin.
  • On laboratory testing, most patients have:
    • Antinuclear autoantibodies
    • Elevated muscle enzymes, notably creatine kinase
  • A portion of patients also develop interstitial lung disease.
  • Amyopathic dermatomyositis (dermatomyositis sinĂ© myositis) is a less common subset which lacks muscle involvement.
  • Polymyositis is a closely related disease with similar, but not identical, muscle pathology, symptoms, treatment, and outcome, but lacks skin involvement.
Epidemiology
Incidence

Incidence varies by ethnic background and location of study:

  • 0.8 per million population studied in Pennsylvania, United States.
  • Female > Male (2:1)
  • Higher incidence among African American women
Risk Factors
  • Susceptibility genes (see genetics)
  • Infectious agents may play a role in the development of dermatolmyositis ...

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FIG. 75.1. Skin changes seen in dermatomyositis. A: Gottron's papules are scaly papules overlying the extensor surfaces of the hands (over the metacarpophalangeal and proximal interphalangeal joints in this case), elbows, knees, or malleoli. This patient also has sclerodactyly and arthritis of the metacarpophalangeal and proximal interphalangeal joints. B: The heliotrope rash is a purplish discoloration around the eyes, especially on the upper lids. C: Linear extensor erythema overlies the extensor surface of the hands beyond the usual location of Gottron's papules or sign. D: Periungual vasculitic changes and cuticular overgrowth. E: Photosensitive diffuse erythroderma with accentuated erythema in the V of the neck (V sign) in a patient with cancer-associated dermatomyositis. F: Drying and cracking of the skin over the lateral and palmar surfaces of the fingers, known as "mechanic's hands," is seen frequently in patients with autoantibodies to aminoacyl-tRNA synthetases (the antisynthetase syndrome).Credit: William J. Koopman, Larry W. Moreland, Arthritis and Allied Conditions A Textbook of Rheumatology, 15e. Philadelphia: Lippincott Williams & Wilkins, 2005.
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