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.

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• Lung Involvement in Destombes-Rosai-Dorfman Disease
  Quentin Moyon, Samia Boussouar, Philippe Maksud, Jean-François Emile, Frédéric Charlotte, Nathalie Aladjidi, Grégoire Prévot, Jean Donadieu, Zahir Amoura, Philippe Grenier, Julien Haroche, Fleur Cohen Aubart
  Chest.2020; 157(2): 323.     CrossRef
• Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature
  Rashid AL Umairi, Danielle Blunt, Wedad Hana, Matthew Cheung, Anastasia Oikonomou
  Case Reports in Radiology.2018; 2018: 1.     CrossRef
• IgG4‐related skin disease may have distinct systemic manifestations: a systematic review
  Adam E. Bennett, Neil A. Fenske, Paul Rodriguez‐Waitkus, Jane L. Messina
  International Journal of Dermatology.2016; 55(11): 1184.     CrossRef
• Nosology and Pathology of Langerhans Cell Histiocytosis
  Jennifer Picarsic, Ronald Jaffe
  Hematology/Oncology Clinics of North America.2015; 29(5): 799.     CrossRef
• A subset of Rosai–Dorfman disease cases show increased IgG4‐positive plasma cells: another red herring or a true association with IgG4‐related disease?
  Madhu P Menon, Moses O Evbuomwan, Juan Rosai, Elaine S Jaffe, Stefania Pittaluga
  Histopathology.2014; 64(3): 455.     CrossRef