Signs & Symptoms

Common characteristics of dermatomyositis in adults and children include proximal muscle weakness, muscle inflammation and a characteristic skin rash.1,2

Onset of dermatomyositis may be acute or insidious, and the rash may accompany or, more commonly, precede the onset of muscle weakness.1-3

The rash commonly occurs in photosensitive areas and is erythematous, oedematous and occasionally pruritic.1

Skin and muscle involvement1,2

Pathognomonic heliotrope rash

  • Red discoloration over the upper eyelids, with or without periorbital oedema
  • Malar and facial rash

Shawl sign

  • Rash on neck, back and shoulders

V-sign

  • Rash on anterior chest

Gottron's sign

  • Gottron’s sign: scaling and erythematous eruptions over metacarpophalangeal and interphalangeal joints1
  • Gottron’s papules or plaques: raised papules or plaques which may accompany Gottron’s sign on the hands. Can also be noted on knees and elbows.1

Symmetric proximal muscle weakness

  • Shoulder, hip girdle limb muscles, truncal muscles1
  • Patients often describe difficulty associated with rising from a low chair, climbing stairs, or raising their arms to lift objects1,2

Juvenile dermatomyositis is characterised by insidious onset of weakness and myalgia following a fever, fatigue or skin rash.1 Patients exhibit irritability and a reluctance to socialise.3 Complications include calcinosis cutis (subcutaneous calcifications), and vasculopathy affecting the gastrointestinal tract.2

Calcinosis cutis is more common in juvenile than adult dermatomyositis.1 Its presence suggests active disease and may be associated with diagnostic and treatment delay.2

Although dermatomyositis is usually painless, pain can be significant in patients with acute disease and calcinosis cutis.2

V-sign (courtesy of Prof. Patrick Cherin)

Common characteristics of dermatomyositis include proximal muscle weakness, muscle inflammation and a characteristic skin rash.1,2

Other complications in dermatomyositis

Other complications in dermatomyositis include malignancy and conditions affecting the joints or the pulmonary, cardiac and gastrointestinal systems.1,2

Systemic complications create challenges for clinicians as patients often have concurrent severe muscle weakness, skin rashes and life-threatening organ involvement, making disease management and treatment more complicated.4

Cardiac

  • While most dermatomyositis patients do not develop cardiac symptoms, arrhythmias or defects in ejection fraction may be present due to involvement of the cardiac muscle in dermatomyositis1
  • Pericarditis, myocarditis and congestive heart failure, despite being rare in dermatomyositis, may lead to increased mortality1

Pulmonary

  • Interstitial lung disease (ILD) is a common pulmonary manifestation of dermatomyositis9
  • ILD manifests as a non-productive cough and dyspnoea, and is the leading cause of death in dermatomyositis patients1
  • Survival is significantly worse for dermatomyositis patients with ILD compared with those without (p=0.02)10
  • DM is also associated with weakness of the ventilatory muscles2
  • There is a strong association between ILD development and MDA-5 autoantibodies11

Gastrointestinal

Due to skeletal and smooth muscle involvement of the gastrointestinal tract in dermatomyositis, complications can occur such as:

  • Dysphagia1,2
  • Impaired gastric motility1
  • Aspiration pneumonia1
  • Bleeding due to vasculopathy of the gut10

Malignancy

  • Risk of malignancy is increased in patients with adult-onset dermatomyositis1,5,6
  • A meta-analysis found that patients with dermatomyositis had a 5.5-fold higher risk for malignancy compared with the general population7
  • Cancers commonly associated with dermatomyositis include ovarian, breast, colon, nasopharyngeal (Asian populations), melanoma, and non-Hodgkin’s lymphoma8
  • Certain autoantibody subtypes are associated with increased risk of malignancy, and these patients should be investigated for malignancies and closely monitored1

Joints

  • Large-joint and small-joint arthralgia are common in dermatomyositis with or without an underlying arthritis1

References

  1. Goyal NA. Continuum (MinneapMinn) 2019; 25:1564-85.
  2. Malik A et al. Front Neurol 2016; 7:64.
  3. Dalakas MC & Hohlfeld R. Lancet 2003; 362:971-82.
  4. Oddis CV & Aggarwal R. Nat Rev Rheumatol 2018; 14:279-89.
  5. Chen Y-J et al. Arthritis Res Ther 2010;12:R70.
  6. Airio A et al. Clin Rheumatol 2006; 25:234-9.
  7. Yang Z et al. J Rheumatol 2015; 42:282-91.
  8. Dalakas MC. N Eng J Med 2015; 372:1734-47.
  9. Morisset J et al. CHEST 2016; 150:1118-28.
  10. Dankó K et al. Medicine 2004; 83:35-42.
  11. Sato S et al. PLoS One 2016; 11:e0154285.

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