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Amyopathic Dermatomyositis

      A 48-year-old man presented with rash, cough, shortness of breath, and arthritis. He had experienced fatigue, fevers, and weight loss of more than 6 months' duration. Examination revealed an increased respiratory rate and hypoxia; fine crackles were audible in both lung bases. Multiple joints were arthritic, but muscle weakness was not clinically evident. This patient had classic findings of dermatomyositis, including a heliotrope rash (Figure 1), Gottron sign (Figure 2), “mechanic's hands” (Figure 3), and the V-sign (Figure 4).
      Figure thumbnail gr1
      FIGURE 1Heliotrope rash is an erythematous rash around the eyes with associated edema.
      Figure thumbnail gr2
      FIGURE 2Gottron sign is evidenced by a scaly raised erythematous rash on the dorsum of the hand on the metacarpophalangeal and proximal interphalangeal joints.
      Figure thumbnail gr3
      FIGURE 3“Mechanic's hands” are characterized by roughening and fissures of skin on the lateral and palmar areas of the fingers.
      Figure thumbnail gr4
      FIGURE 4The V-sign is violaceous erythema on the chest and neck.
      Dermatomyositis is an idiopathic microangiopathic disorder that affects the skin and muscles. It is thought to be caused by deposition of complement in the blood vessels.
      • Dalakas M
      • Hohlfeld R
      Polymyositis and dermatomyositis.
      Most patients experience symmetric proximal muscle weakness. The diagnosis is made by typical skin findings and evidence of muscle injury.
      • Dalakas M
      • Hohlfeld R
      Polymyositis and dermatomyositis.
      Notably, this patient had a variant of dermatomyositis called amyopathic dermatomyositis, which manifests without muscle involvement.
      • Euwer RL
      • Sontheimer RD
      Amyopathic dermatomyositis: a review.
      He also had interstitial lung disease, which has been associated with the amyopathic variant as well as with classic dermatomyositis.
      • Marie I
      • Hachulla E
      • Chérin P
      • et al.
      Interstitial lung disease in polymyositis and dermatomyositis.
      Presented as the “Mayo Medical Image” for Grand Rounds on September 22, 2010, Mayo Clinic, Rochester, MN.

      REFERENCES

        • Dalakas M
        • Hohlfeld R
        Polymyositis and dermatomyositis.
        Lancet. 2003; 362: 971-982
        • Euwer RL
        • Sontheimer RD
        Amyopathic dermatomyositis: a review.
        J Invest Dermatol. 1993; 100: S124-S127
        • Marie I
        • Hachulla E
        • Chérin P
        • et al.
        Interstitial lung disease in polymyositis and dermatomyositis.
        Arthritis Rheum. 2002; 47: 614-622