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.

Citations

Citations to this article as recorded by  

• 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
• Pulmonary IgG4+ Rosai-Dorfman disease
  Karim El-Kersh, Rafael L Perez, Juan Guardiola
  BMJ Case Reports.2013; 2013: bcr2012008324.     CrossRef

Rosai-Dorfman disease (RDD) can present in any anatomic site, but breast involvement is rarely reported. Recently, a relationship between RDD and IgG4-related sclerosing disease has been suggested. Here we report another case of RDD with overlapping features of IgG4-related sclerosing disease occurring in a right breast of a 62-year-old female. On microscopic examination, the mass demonstrated a characteristic zonal pattern of proliferation of large polygonal histiocytes and lymphoplasma cells with stromal fibrosis. Emperipolesis was observed in histiocytes with abundant cytoplasm, which showed immunoreactivity for S-100 protein and CD68; the diagnosis of RDD was made. Sheets of plasma cells in the fibrotic stroma demonstrated positive reactions for IgG and IgG4. The mean count of IgG4-positive plasma cells was 100.2/high power field, and the ratio of IgG4/IgG was 56.7%. Additional findings of stromal fibrosis and obliteration of preexisting breast lobules suggested overlapping features with IgG4-related sclerosing disease.

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• Extranodal Rosai–Dorfman Disease With Increased IgG4-Positive Plasma Cells Involving the Breast: A Case Report With Review of the Literature
  Raymond Chimatira, Raisa Wessels
  The American Journal of Dermatopathology.2026; 48(5): 391.     CrossRef
• From Tumor Suspicion to Immune Revelation: A Breast Lump Signals IgG4-related Disease
  Renu Singh, Gurwinder Kaur, Md Ali Osama, Chitresh Kumar, Devender Bairwa
  Indian Journal of Gynecologic Oncology.2026;[Epub]     CrossRef
• Extranodal Rosai–Dorfman Disease of the Male Breast: A Case Report and Literature Review
  Raymond Chimatira, Dharshnee R. Chetty
  The American Journal of Dermatopathology.2026; 48(6): 452.     CrossRef
• Primary Cutaneous Rosai–Dorfman Disease Presenting as an Infiltrative Dorsal Nasal Mass With Deep Skeletal Muscle Involvement
  Luke Hamilton, Julien C. Bourgeois, Katherine Johnson, Antonio Jimenez, Janice M. Wilson, Brandon P. Goodwin
  The American Journal of Dermatopathology.2026;[Epub]     CrossRef
• Rosai-Dorfman Disease of the Breast: Radiologic-Pathologic Correlation
  Kathryn W Zamora, Stefanie Zalasin, Ami Desai, Hua Guo
  Journal of Breast Imaging.2025; 7(5): 576.     CrossRef
• Diagnosis and management of Rosai–Dorfman disease of the breast: Case report and literature review
  Jessman King Lun Lo, Sara Wai Wun Fung, Zara Chui San Tsang
  Surgical Practice.2024; 28(4): 229.     CrossRef
• IgG4-related Breast Disease: Review of the Literature
  Helana Jeries, Yolanda Braun-Moscovici, Alexandra Balbir-Gurman
  Rambam Maimonides Medical Journal.2024; 15(4): e0018.     CrossRef
• Pathology of IgG4-related sclerosing mastitis
  Polycarp Erivwo, Gulisa Turashvili
  Journal of Clinical Pathology.2021; 74(8): 475.     CrossRef
• Rosai–Dorfman Disease: Breast Involvement—Case Report and Literature Review
  George Iancu, Nicolae Gica, Laura Mihaela Mustata, Anca Maria Panaitescu, Danut Vasile, Gheorghe Peltecu
  Medicina.2021; 57(11): 1167.     CrossRef
• Sonographic features of Rosai‐Dorfman disease in the breast: A case report
  Gi W. Shin, Young M. Park, Young J. Heo, Jin W. Baek, Yoo J. Lee, Ji Y. Han, Hayoung Park
  Journal of Clinical Ultrasound.2020; 48(2): 108.     CrossRef
• Rosai-Dorfman Disease of the Breast With Variable IgG4+ Plasma Cells
  Jenny C. Hoffmann, Chieh-Yu Lin, Siddhartha Bhattacharyya, Olga K. Weinberg, Karen M. Chisholm, Michael Bayerl, Michael Cascio, Girish Venkataraman, Kimberly Allison, Megan Troxell, Chung-Che Chang, Adam Bagg, Tracy I. George, Dennis O’Malley, Robert S. O
  American Journal of Surgical Pathology.2019; 43(12): 1653.     CrossRef
• Rosai-Dorfman disease presenting as a breast mass
  Ding Dai, Qi Cai, Nasreen A. Vohra, Jan Wong, Zsuzsanna P. Therien, Karlene Hewan-Lowe, Ann Sutton
  Archives of Pathology and Clinical Research.2019; 3(1): 008.     CrossRef
• Erdheim-Chester Disease Involving Lymph Nodes and Liver Clinically Mimicking Lymphoma: A Case Report
  Yeoun Eun Sung, Yoon Seo Lee, Jieun Lee, Kyo Young Lee
  Journal of Pathology and Translational Medicine.2018; 52(3): 183.     CrossRef
• Enfermedad de Rosai-Dorfman en mama de paciente masculino: una entidad rara
  Paola Iturralde Rosas-Priego, José Daniel Flores-Alatriste, Daniela Stuht López, Javier Gómez Pedroso-Rea, Cecilia Ortiz-de-Iturbide, Jorge Valenzuela-Tamariz, Manuel Ubiergo-García
  Revista de Senología y Patología Mamaria.2018; 31(2): 72.     CrossRef
• Increased Immunoglobulin G4-positive Plasma Cells in Lymphadenoma of the Salivary Gland: An Immunohistochemical Comparison Among Lymphoepithelial Lesions
  Jiyoon Kim, Joon Seon Song, Jong-Lyel Roh, Seung-Ho Choi, Soon Yuhl Nam, Sang Yoon Kim, Kyung-Ja Cho
  Applied Immunohistochemistry & Molecular Morphology.2018; 26(6): 420.     CrossRef
• IgG4-Related Sclerosing Disease of the Breast in a Male Patient
  Taisia Vitkovski, Galina S. Marder, Dominic A. Filardi, Ekta Gupta, Frank Breuer
  International Journal of Surgical Pathology.2017; 25(8): 711.     CrossRef
• Extranodal manifestation of Rosai-Dorfman disease in the breast tissue
  Qiao Zhou, Umer Ansari, Nandan Keshav, Fiona Davis, Maria Cundiff
  Radiology Case Reports.2016; 11(3): 125.     CrossRef
• Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
  Han Ma, Yue Zheng, Guoxing Zhu, Jie Wu, Chun Lu, Wei Lai
  Annals of Dermatology.2015; 27(1): 71.     CrossRef
• IgG4-related disease of the breast: a systemic disease whose mammary manifestations mimic breast cancer
  Takuya Moriya, Hisashi Hirakawa, Maki Nagashima, Mitsuhiko Yasuda, Izo Kimijima
  International Cancer Conference Journal.2015; 4(2): 67.     CrossRef
• Primary Cutaneous Marginal IgG4 Lymphoma and Rosai–Dorfman's Disease Coexisting in Several Lesions of the Same Patient
  Salma Machan, Camino Medina, Socorro María Rodríguez-Pinilla, José M. Suárez-Peñaranda, Yolanda Castro, Paula Molés, Celia Requena, Carles Saus, Luis Requena, Carlos Santonja
  The American Journal of Dermatopathology.2015; 37(5): 413.     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
• Freiburg Neuropathology Case Conference: A Partially Calcified, Dura-based Tumour of the Frontal Lobe
  C. A. Taschner, O. Staszewski, R. Jabbarli, A. Keuler, M. Prinz
  Clinical Neuroradiology.2013; 23(1): 63.     CrossRef
• Pulmonary IgG4+ Rosai-Dorfman disease
  Karim El-Kersh, Rafael L Perez, Juan Guardiola
  BMJ Case Reports.2013; 2013: bcr2012008324.     CrossRef