Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report

Rosai-Dorfman disease of the oral cavity

Abir Charfeddine; Mounir Omami; Marwa Garma; Ahlem Bellalah; Sameh Sioud; Jamil Selmi

Downloads: 0
Views: 270


First described by J Rosai and R F Dorfman in 1969, Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. It is usually seen in the first two decades of life. The most frequent clinical presentation is painless, bilateral cervical lymphadenopathy accompanied by fever, weight loss, and an elevated ESR. However, RDD without nodal involvement is extremely rare, and the most common extranodal location is the head and neck region, mainly affecting the nasal cavity, pharynx, and paranasal sinuses. Oral location of RDD is occasional; according to our knowledge, only 17 cases of oral Rosai-Dorfman disease without lymph node involvement have been found in the literature. Because of the rarity of these isolated oral presentations, the clinical and radiological aspects need to be more studied. This article aims to present a rare case of oral Rosai-Dorfman disease without nodal involvement, detail the clinical and radiological signs, and the treatment strategy used in our patient.


Emperipolesis, Histiocytosis, Sinus, Lymph Nodes, Maxilla


1 Bruce-Brand C, Schneider JW, Schubert P. Rosai-Dorfman disease: an overview. J Clin Pathol. 2020;73(11):697-705. PMid:32591351.

2 Hamza A, Zhang Z, Al-Khafaji B. Solitary extranodal rosai-dorfman disease of the mandible: an exceedingly rare presentation. Autops Case Rep. 2018;8(3):e2018036. PMid:30101140.

3 Güven G, Ilgan S, Altun C, Gerek M, Gunhan O. Rosai–Dorfman disease of the parotid and submandibular glands: salivary gland scintigraphy and oral findings in two siblings. Dentomaxillofac Radiol. 2007;36(7):428-33. PMid:17881605.

4 Yaman B, Cerit L, Kemal HS, Usalp S, Duygu H. Unusual involvement of right ventricle in patient with Rosai–Dorfman disease. Anatol J Cardiol. 2019;22(5):271.

5 Chen TD, Lee LY. Rosai-dorfman disease presenting in the parotid gland with features of IgG4-related sclerosing disease. Arch Otolaryngol Neck Surg. 2011;137(7):705-8. PMid:21502465.

6 Goodnight JW, Wang MB, Sercarz JA, Fu YS. Extranodal rosai-dorfman disease of the head and neck. Laryngoscope. 1996;106(3):253-6. PMid:8614184.

7 Kasper HU, Hegenbarth V, Buhtz P. Rhinoscleroma associated with Rosai-Dorfman reaction of regional lymph nodes. Pathol Int. 2004;54(2):101-4. PMid:14720140.

8 Tekin U, Tüz HH, Günhan Ö. Reconstruction of a patient with rosai-dorfman disease using ramus graft and osseointegrated implants: a case report. J Oral Implantol. 2012;38(1):79-83. PMid:20553156.

9 Ojha J, Rawal YB, Hornick JL, et al. Extra nodal rosai-dorfman disease originating in the nasal and paranasal complex and gnathic bones: a systematic analysis of seven cases and review of literature. Head Neck Pathol. 2020;14(2):442-53. PMid:31368076.

10 Wenig BM, Abbondanzo SL, Childers EL, Kapadia SB, Heffner DR. Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck. Hum Pathol. 1993;24(5):483-92. PMid:8491488.

11 Kademani D, Patel SG, Prasad ML, Huvos AG, Shah JP. Intraoral presentation of Rosai-Dorfman disease: a case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002;93(6):699-704. PMid:12142877.

12 Keskin A, Genç F, Günhan OJ. Rosai-Dorfman disease involving maxilla: a case report. J Oral Maxillofac Surg. 2007;65(12):2563-8. PMid:18022485.

13 Demicco EG, Rosenberg AE, Björnsson J, Rybak LD, Unni KK, Nielsen GP. Primary rosai-dorfman disease of bone: a clinicopathologic study of 15 cases. Am J Surg Pathol. 2010;34(9):1324-33. PMid:20679880.

14 Keskin A, Genç F, Günhan Ö. Rosai-dorfman disease involving maxilla: a case report. J Oral Maxillofac Surg. 2007;65(12):2563-8. PMid:18022485.

15 Koslovsky DA, Alfi DM, Pradhan JS, Philipone EM, Kelsch RD, Bastidas JA. Oral cavity rosai-dorfman disease without lymphadenopathy: a case series from two institutions within the metropolitan new york area. J Oral Maxillofac Surg. 2012;70(9):e75.

16 Alencar FSL, Soares ÁC, Stahel-Lage LF, Menezes Pontes JR. Oral signs in a patient with undiagnosed rosai-dorfman disease: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol. 2020;129(1):e93.

17 Alawi F, Robinson B, Carrasco L. Rosai-Dorfman disease of the mandible. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontology. 2006;102(4):506-12. PMid:16997119.

18 Ojha J, McIlwain R, Said-Al Naief N. A large radiolucent lesion of the posterior maxilla. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontology. 2010;110(4):423-9. PMid:20868993.

19 Dickson-González SM, Rodríguez-Morales AJ, Jiménez L, et al. Maxillofacial Rosai–Dorfman disease in a newly diagnosed HIV-infected patient. Int J Infect Dis. 2008;12(2):219-21. PMid:17714970.

20 Miniello TG, Araujo JP, Sugaya NN, Elias FM, Almeida OPD, Alves FA. Rosai-Dorfman disease affecting the maxilla. Autops Case Rep. 2016;6(4):49-55. PMid:28210574.

21 Kaltman JM, Best SP, McClure SA. Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman disease: a unique case presentation. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011;112(6):e124-6. PMid:21906971.

22 Petrovic D, Mihailovic D, Petrovic S, et al. Asymptomatic flow of Rosai-Dorfman disease. Vojnosanit Pregl. 2014;71(8):780-3. PMid:25181840.

23 American Society for Dermatologic Surgery. Successful treatment of cutaneous rosai–dorfman disease with pulsed dye laser. Dermatol Surg. 2019;00:1-3.

Submitted date:

Accepted date:

Publication date:

656f35dca95395275f03aa92 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections