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정상 Creatine Kinase를 보인 조직학적으로 증명된 피부근염 1예

Biopsy-Proven Dermatomyositis with Normal Creatine Kinase

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초록

피부근염은 특발성 염증성 근병증으로 골격근과 피부의 염증성 침윤을 특징으로 한다[1]. 염증성 근염과 관련된 근육효소 가운데 creatine kinase (CK)는 임상에서 가장 유용하게 사용되고 있는데 이는 CK가 근육 손상에 가장 민감하고 다른 근육효소에 비해 상승의 폭이 더 크기 때문이다[2]. Bohan 등[3]은 153명의 염증성 근염 환자 중 혈중 CK가 상승하지 않은 염증성 근염은 4.6%로 보고하였으나, 국내에서는 현재까지 조직학적으로 증명된 피부근염 환자에서 정상 범위의 CK를 보인 증례는 보고된 바 없다.

Abstract

Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) characterized by skeletal muscle inflammation and typical skin manifestations. Creatine kinase (CK) has traditionally been considered to be the most useful serum enzyme for the diagnosis and assessment of adult patients with IIM. To our knowledge, there has been no reported case of biopsy-proven DM without CK elevation in Korea, to date. Panniculitis is an uncommon cutaneous manifestation in adult patients with DM. A search of the PubMed database reveals fewer than 30 reported cases of panniculitis in adult patients with DM. Here, we report a case of a 42-year-old female who was diagnosed with biopsy-proven DM with normal serum CK levels and panniculitis.

참고문헌 (10)

  1. Iaccarino L, "The clinical features, diagnosis and classification of dermatomyositis", J Autoimmun, 48-49, pp.122~127 (2014)
  2. Tarqoff IN, "Laboratory testing in the diagnosis and management of idiopathic inflammatory myopathies", Rheum Dis Clin North Am, 28, pp.859~890 (2002)
  3. Bohan A, "Computerassisted analysis of 153 patients with polymyositis and dermatomyositis", Medicine(Baltimore), 56, pp.255~286 (1977)
  4. Eberhard BA, "Panniculitis and lipodystrophy", Curr Opin Rheumatol, 14, pp.566~570 (2002)
  5. Bohan A, "Polymyositis and dermatomyositis(first of two parts)", N Engl J Med, 292, pp.344~347 (1975)
  6. Fudman EJ, "Dermatomyositis without creatine kinase elevation. A poor prognostic sign", Am J Med, 80, pp.329~332 (1986)
  7. Nozaki K, "High aldolase with normal creatine kinase in serum predicts a myopathy with perimysial pathology", J Neurol Neurosurg Psychiatry, 80, pp.904~908 (2009)
  8. Lee YH, "Dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia", Korean J Intern Med, 15, pp.85~88 (2000)
  9. Tateyama M, "Clinical values of FDG PET in polymyositis and dermatomyositis syndromes : imaging of skeletal muscle inflammation", BMJ Open, 5, pp.e006763~ (2015)
  10. Cantwell C, "A comparison of inflammatory myopathies at whole-body turbo STIR MRI", Clin Radiol, 60, pp.261~267 (2005)

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