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Case Reports
. 2014 Feb 28;7(2):68-72.
doi: 10.4066/AMJ.2014.1931. eCollection 2014.

Rosai-Dorfman disease: A rare clinico-pathological presentation

Bhushan M Warpe  1 , Shraddha V More  2
Affiliations
Case Reports

Rosai-Dorfman disease: A rare clinico-pathological presentation

Bhushan M Warpe et al. Australas Med J. .

Abstract

Rosai-Dorfman disease (RDD) is also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML). It is a rare, benign, self-limiting disease of phagocytic histiocytes affecting a young age group presenting with massive painless cervical lymphadenopathy. RDD has nodal and also extra-nodal involvement with episodes of exacerbation and remissions, whose aetiology remains poorly elucidated. It is highly variable in its clinical presentation and response to treatment. Its treatment is poorly defined but the prognosis is usually favourable. Here we are reporting a rare, unusual clinical presentation of cervical lymphadenopathy and nasal mass diagnosed as RDD with cyto-histopathological correlation. Only a few such cases have been reported in the literature. We also emphasise that clinicians and pathologists should always be aware of RDD in making a differential diagnosis of cervical lymphadenopathy.

Keywords: Rosai-Dorfman Disease; cervical lymphadenopathy; emperipolesis; histiocytes.

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Conflict of interest statement

CONFLICTS OF INTEREST

The authors declare that they have no competing interests.

Figures

Figure 1
Figure 1. Clinical photograph-Enlarged left cervical lymph node (level III)
Figure 2
Figure 2. Clinical photograph-Bilateral anterior nasal mass partially obstructing the anterior nasal nares
Figure 3
Figure 3. Microphotograph-FNAC smear showing large histiocytes with ‘emperipolesis’. (MGG, x 400)
Figure 4
Figure 4. Microphotograph-Tissue section from cervical lymph node showing partial effacement of its architecture with prominent dilated lymph sinuses. (H&E, x40)
Figure 5
Figure 5. Microphotograph—Tissue section from cervical lymph node showing large histiocytes with ‘emperipolesis’ in lymph sinuses. (H&E, x 400)
Figure 6
Figure 6. Microphotograph-Tissue section showing ulcerated nasal mucosa with lymphoplasmacytic infiltrate and few histiocytes with ‘emperipolesis’ in sub-epithelial tissue. (H&E, x 400)
See this image and copyright information in PMC

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