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Rosai-Dorfman Disease: Report of a Case Associated with IgG4-Related Sclerotic Lesions

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We describe a rare case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) associated with a six-year history of autoimmune pancreatitis, which was controlled by steroid treatment. The patient presented with multiple, cervical and thoracic lymphadenopathy and abnormal, nodular opacities in the lung. Histologically, Rosai-Dorfman disease with numerous IgG4-positive cells was identified in a subcutaneous lymph node in the patient’s left forearm. The patient recovered uneventfully with steroid treatment.

참고문헌 (9)

  1. Gaitonde S, "Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: an overview", Arch Pathol Lab Med, 131, pp.1117~1121 (2007)
  2. Roberts SS, "IgG4+ Rosai-Dorfman disease of the lung", Histopathology, 56, pp.662~664 (2010)
  3. Richter JT, "Extranodal Rosai-Dorfman disease presenting as a cardiac mass in an adult: report of a unique case and lack of relationship to IgG4-related sclerosing lesions", Hum Pathol, 41, pp.297~301 (2010)
  4. Kuo TT, "IgG4-positive plasma cells in cutaneous Rosai-Dorfman disease: an additional immunohistochemical feature and possible relationship to IgG4-related sclerosing disease", J Cutan Pathol, 36, pp.1069~1073 (2009)
  5. Morkowski JJ, "Rosai-Dorfman disease confined to the breast", Ann Diagn Pathol, 14, pp.81~87 (2010)
  6. Shrestha B, "Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis: report of 6 and 12 cases with similar histopathology", Am J Surg Pathol, 33, pp.1450~1462 (2009)
  7. Cheuk W, "IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity", Adv Anat Pathol, 17, pp.303~332 (2010)
  8. Zhang L, "Autoimmune pancreatitis and IgG4-related systemic diseases", Int J Clin Exp Pathol, 3, pp.491~504 (2010)
  9. Sato Y, "IgG4-related disease: historical overview and pathology of hematological disorders", Pathol Int, 60, pp.247~258 (2010)

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