A Case of Dermatomyositis with Mechanic's Hands, Gottron's Signs, and Anti-140kDa Antibody: Rapid Fatal Course due to Rapidly Progressive Interstitial Pneumonia

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  • Ｍｅｃｈａｎｉｃ’ｓ　Ｈａｎｄ，逆Ｇｏｔｔｒｏｎ徴候を呈し，急速進行性間質性肺炎により死の転帰をとった抗１４０ｋＤａ蛋白抗体陽性の皮膚筋炎の１例
  • 症例 Mechanic's Hand,逆Gottron徴候を呈し,急速進行性間質性肺炎により死の転帰をとった抗140kDa蛋白抗体陽性の皮膚筋炎の1例
  • ショウレイ Mechanic s Hand ギャクGottron チョウコウ オ テイシ キュウソク シンコウセイ カンシツセイ ハイエン ニ ヨリ シ ノ テンキ オ トッタ コウ140kDa タンパク コウタイ ヨウセイ ノ ヒフ キンエン ノ 1レイ

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A-57-year-old woman showed skin lesions characteristic of dermatomyositis : Gottron's signs, mechanic's hands, erythematous plaques on her arms and legs, edema of her eyelids, and edematous erythema of the back, with general malaise and dry cough. Muscle weakness was not observed. Laboratory findings and MRI scan and electromyogram revealed very slight myositis. The patient received a clinical diagnosis of amyopathic dermatomyositis (CADM). She also had rapidly progressive interstitial pneumonia. Pulse therapy with methylprednisolone (1 g × 3 days) and administration of oral prednisolone in combination with cyclosporine A and intravenous cyclophosphamide could not improve her condition, and she died of respiratory failure only one month later. Immunoprecipitation studies revealed a positive reaction for an anti-140kDa antibody, which suggested anti-CADM 140 antibody. An association of mechanic's hands with Gottron's signs on flexor surface of fingers and interstitial pneumonia was presumed. In conclusion, a patient with CADM and rapidly progressive interstitial pneumonia should have been treated with intensive immunosuppressants as soon as possible to prevent a worse prognosis.

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