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Rosai Dorfman Disease of the Orbit

Geetha Narayanan, Pranab Prabhakaran, Priya Mary Jacob, Rekha Nair, Ani Sreedhar

Abstract


 Introduction: Rosai-Dorfman disease or Sinus Histiocytosis with massive lymphadenopathy (SHML) is a rare, benign and self-limited histiocytic proliferative disorder which affects mainly lymph nodes and very rarely extranodal sites.

Presentation of case: A 60 year old lady with SHML of orbit is described. She underwent excision of the mass, received steroids for 6 weeks and is in remission at 2 years.

Conclusion: Though the orbit is a rare site for SHML, the disease should be considered in the differential diagnosis of orbital swellings.

 


Keywords


SHML; Rosai Dorfman; Orbit

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References


Pivetti-Pezzi P, Torce C, Colabelli-Gisoldi RA, Vitale A, Baccari A, Pacchiarotti A. Relapsing bilateral uveitis and papilledema in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Eur J Ophthalmol. 1995, 5:59-62

Goldberg S, Mahadevia P, Lipton M, Rosenbaum PS. Sinus histiocytosis with massive lymphadenopathy involving the orbit: reversal of compressive optic neuropathy after chemotherapy. J Neuroophthalmol. 1998, 18:270-275

Rumelt S, Cohen I, Rehany U. Marginal corneal infiltrates: a possible new manifestation of sinus histiocytosis with massive lymphadenopathy. Cornea. 2000, 19:857-858

R Khan, P Moriarty S Kennedy. Rosai Dorfman disease or sinus histiocytosis with massive lymphadenopathy of the orbit. Br J Ophthalmol. 2003, 87:1043-1055


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