Anti-Mi-2 and Anti-TIF1-<i>γ</i> Double-Positive Juvenile Dermatomyositis Treated under Diagnosis of Chronic Eczema: A Case Report

  • Yoshida Shuhei
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Matsumoto Haruki
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Fujita Yuya
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Yokose Kohei
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Temmoku Jumpei
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Matsuoka Naoki
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Yashiro-Furuya Makiko
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Asano Tomoyuki
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Sato Shuzo
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Suzuki Eiji
    Department of Rheumatology, Ohta Nishinouchi General Hospital Foundation
  • Yago Toru
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Yaguchi Takae
    Department of General Internal Medicine, Fukushima Medical University School of Medicine
  • Aita Tetsuro
    Department of General Internal Medicine, Fukushima Medical University School of Medicine
  • Kusano Misaki
    Department of Dermatology, Fukushima Medical University School of Medicine
  • Yamamoto Toshiyuki
    Department of Dermatology, Fukushima Medical University School of Medicine
  • Watanabe Hiroshi
    Department of Rheumatology, Fukushima Medical University School of Medicine
  • Migita Kiyoshi
    Department of Rheumatology, Fukushima Medical University School of Medicine

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<p>Myositis-specific autoantibodies are relevant factors that define the disease phenotype of dermatomyositis (DM). Anti-Mi-2 antibody-positive DM patients may present with the typical skin lesions and prominent myositis. On the other hand, adult DM patients with anti-TIF-γ antibody seem to be associated with internal malignancy. Here, we report a rare case of juvenile dermatomyositis (JDM) exhibiting anti-Mi-2 and anti-transcriptional intermediary factor-1 gamma (TIF1-γ) antibodies, with no internal malignancy. A 16-year-old female Japanese patient under treatment with a 2-year history of chronic eczematous lesions was admitted to our department with elevated levels of muscle enzymes. Characteristic skin changes, such as Gottron’s papules of the hand, heliotrope rash of the eyelids, and poikiloderma-like legions and diffuse pigmentation on the back, were observed. Histologically, the patient’s skin was characterized by the presence of lymphocytic vascular inflammation and endothelial swelling, which are consistent with DM. Severe symmetric proximal muscle weakness, elevated serum muscle enzymes and the presence of anti-TIF1-γ and Mi-2 antibodies were noted. The diagnosis of JDM was made according to the European League Against Rheumatism (EULAR) diagnostic criteria. A high dose of corticosteroids and following intravenous cyclophosphamide treatment (750 mg three times) resulted in an improvement in clinical manifestations and functional outcomes, and recurrence did not occur. Estimation of autoantibodies may serve as an ancillary tool in delineating and defining distinct clinical phenotypes in JDM.</p>

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