Rosai-Dorfman disease: A case report

How to cite this article:
Swami SY, Lagdive PM, Gadkari RU. Rosai-Dorfman disease: A case report. Ann Trop Med Public Health 2015;8:40-2
How to cite this URL:
Swami SY, Lagdive PM, Gadkari RU. Rosai-Dorfman disease: A case report. Ann Trop Med Public Health [serial online] 2015 [cited 2016 Sep 14];8:40-2. Available from:

Dear Sir,

Rosai-Dorfman Disease (RDD) is a rare disease of histiocyte proliferation. It predominantly affects children and young adults, with a slight male preponderance. [1],[2] Though the disease has the capacity to simulate a malignant process both clinically and pathologically, it has a benign clinical course.

A 25-year-old male presented with bilateral multiple cervical and submandibular swellings associated with fever since the past 5-6 months.

On examination, bilateral cervical and submandibular swellings of sizes ranging from 1 × 0.5 cm to 5 × 3 cm were found [Figure 1] and [Figure 2]. They were firm in consistency, nontender, and freely mobile. Peripheral smear examination was within normal limits. Fine-needle aspiration cytology (FNAC) revealed plenty of mature and transforming lymphocytes, plasma cells, and histiocytes showing emperipolesis (intact lymphocytes in the cytoplasm) [Figure 3] and [Figure 4].

Figure 1: Showing left cervical and submandibular swellings

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Figure 2: Showing right cervical and submandibular swellings

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Figure 3: Microphotograph showing lymphocytes, plasma cells, and histiocytes showing emperipolesis (Pap: 20×)

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Figure 4: Microphotograph showing histiocyte showing emperipolesis (Pap: 40×)

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Differential diagnoses include Langerhans cell histiocytosis (LCH), reactive lymph node hyperplasia with sinus histiocytosis (RLHSH), and hemophagocytic syndrome [Table 1].

Table 1: Differential diagnoses of RDD
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Rosai-Dorfman disease (RDD) is a rare, benign, idiopathic, proliferative disease of phagocytic histiocytes. The etiology and pathogenesis of this disorder are unknown but are thought to represent a reactive histiocytic process to an infective agent rather than a neoplastic or other primary condition. [3]

The etiological agent is thought to be stimulation of monocyte/macrophage via macrophage colony-stimulating factor (M-CSF). [4]

The most common presentation of RDD is massive painless bilateral lymphadenopathy with fever, as in our case, and the most commonly involved site is a cervical lymph node. Extranodal involvement occurs in 30-40% of cases, most often in the head and neck. [5]

Immunohistochemistry results were: Cluster designation 68 (CD68) + ve, S100 +ve, and CD 1a -ve. [6] Cytodiagnosis is an important tool in RDD, as in our case.

Other associated conditions are malignant lymphoma, non-Hodgkin lymphoma, myeloma, melanoma, papillary carcinoma of thyroid, and immature teratoma of ovary. [7]

Mostly, the lymph nodes regress by themselves; however, some patients may require surgical debulking, and, rarely, radiotherapy and chemotherapy. [8]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.
Paulli M, Bergamaschi G, Tonon L, Viglio A, Rosso R, Facchetti F, et al. Evidence for a polyclonal nature of cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Br J Haematol 1995;91:415-8.
Grabczynska SA, Toh CT, Francis N, Costello C, Bunker CB. Rosai-Dorfman disease complicated by autoimmune haemolytic anemia: Case report and review of a multisystem disease with cutaneous infiltrates. Br J Dermatol 2001;145:323-6.
Middel P, Hemmerlein B, Fayyazi A, Kaboth U, Radzun HJ. Sinus histiocytosis with massive lymphadenopathy: Evidence for its relationship to macrophages and for a cytokine-related disorder. Histopathology 1999;35:523-33.
Perry BP, Gregg CM, Myers S, Lilly S, Mann KP, Prieto V. Rosai-Dorfman disease (extranodal sinus histiocytosis) in a patient with HIV. Ear Nose Throat J 1998;77:855-8.
Ahsan SF, Madgy DN, Poulik J. Otolaryngologic manifestation of Rosai-Dorfman’s Disease. Int J Pediatr Otorhinolaryngol 2001;59:221-7.
Lopes LF, Bacchi MM, Coellho KI, Filho AA, Bacchi CE. Emperipolesis in a case of B-cell lymphoma: A rare phenomenon outside of Rosai-Dorfman disease. Ann Diagn Pathol 2003;7:310-3.
Hameed S, Husain A, Riaz S. Rosai Dorfman’s disease. Ann Punjab Med Coll 2008;2:65-68.

Source of Support: None, Conflict of Interest: None

Read associated Letter to the Editor: Rosai-Dorfman disease: A case report with this article

DOI: 10.4103/1755-6783.157289


[Figure 1], [Figure 2], [Figure 3], [Figure 4]


[Table 1]

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