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Extra Nodal Rosai–Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) Presenting as Asymmetric Bilateral Optic Atrophy

An Atypical Ocular Presentation

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Abstract

Rosai–Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder, presenting as painless bilateral cervical lymphadenopathy, with systemic symptoms. Extra nodal manifestations have been reported in 28–43 % cases with rare ocular involvement. We report a case of a 57 year old female presenting with gradual progressive decrease of vision OU since 8 months associated with epistaxis. Fundus examination revealed established optic atrophy in right eye with features of chronic papilloedema in left eye suggestive of compressive lesion. CT of brain, paranasal sinuses confirmed the presence of homogenously enhancing mass in left ethmoid sinus, left sphenoid sinus extending into suprasellar region. The biopsy of this mass revealed extra nodal SHML with tissue sections being S100 and CD68 positive with emperipolesis noted. Here we describe this atypical ocular presentation of extra nodal SHML to highlight that this rare disease can manifest as an aggressive sight threatening entity, even in older age group.

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Acknowledgments

This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Authors’ Contribution

All authors have contributed equally to the varied aspects of this case report such as data collection, patient treatment, literature review and final editing of the manuscript.

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Authors and Affiliations

  1. Department of Ophthalmology, T.N.M.C & B.Y.L Nair Charitable Hospital, Mumbai, India

    Eesha Shukla, Anjali Nicholson, Anamika Agrawal & Darshana Rathod

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  1. Eesha Shukla
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  2. Anjali Nicholson
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  3. Anamika Agrawal
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  4. Darshana Rathod
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Correspondence to Eesha Shukla.

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This work presented above complies with the guidelines for human studies and animal welfare regulations. The subject has given their informed consent and as this was a case report, the institute’s ethics committee has allowed the case report without a study protocol. No animal experimentation was done during the course of this work. IRB/Ethics Committee had ruled that approval was not required for the study.

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Shukla, E., Nicholson, A., Agrawal, A. et al. Extra Nodal Rosai–Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) Presenting as Asymmetric Bilateral Optic Atrophy. Head and Neck Pathol 10, 414–417 (2016). https://doi.org/10.1007/s12105-016-0719-4

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  • DOI: https://doi.org/10.1007/s12105-016-0719-4

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