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A Bad Sign: Dermatomyositis with Interstitial Lung Disease

(A) A clinical examination revealed confluent symmetric violaceous patches on the malar eminences. (B) Additional violaceous patches were evident on the metacarpophalangeal joints. (C) Tender, erythematous papules marked the patient’s palms. (D) An ulcer (arrow) was noted on the antihelix. (E) A papule with a central ulcer was seen on the elbows.

The presence of an autoantibody explained why a patient with dermatomyositis had respiratory symptoms. A 52-year-old man presented with a 1-month history of fever and rash, and a 2-week history of muscle pain and muscle weakness, which made it difficult for him to rise from a sitting position. The patient denied being a smoker or taking any medicine regularly.

A physical examination revealed coarse crepitation in both lungs and proximal muscle weakness, grades III/IV. He had confluent, symmetric, violaceous patches on his scalp, malar eminences, the “V” area of the upper chest, the upper back, the third and fourth metacarpophalangeal joints, and the extensor tendons of both hands (Figures 1A and B). Multiple discrete, dull, erythematous papules marked his palms and fingers, and he had papules with central ulcers on the antihelices of his auricles and on his elbows (Figures 1C-E). Five days after admission to the hospital, his respiratory function began to decline. He developed progressive dyspnea with oxygen desaturation.

Assessment

Laboratory testing disclosed a creatine phosphokinase level of 1109 U/L (normal, 30-190 U/L) and an antinuclear antibody titer of 1:80 in a nucleolar pattern. Noncontrast computed tomography (CT) of the chest revealed predominant ground-glass opacity with traction bronchiectasis in both lower lobes (Figure 2). Anti-melanoma differentiation-associated gene 5 (MDA-5) autoantibody was detected in the patient’s serum.

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-Siriwan Palawisuth, MDa,1, Kornphaka Kantikosum, MDa,1, Mattana Patiyasikunt, MDa, Thitiwat Sriprasart, MDb, Pravit Asawanonda, MD, DSca, Pawinee Rerknimitr, MD, MSca

This article originally appeared in the February issue of The American Journal of Medicine.

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