title: Extra Nodal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) Presenting as Asymmetric Bilateral Optic Atrophy : An Atypical Ocular Presentation.
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.
authors.affiliation: Department of Ophthalmology, T.N.M.C & B.Y.L Nair Charitable Hospital, Mumbai, India. eesha3388@gmail.com.
authors.forename: Eesha
authors.initials: E
authors.lastname: Shukla
authors.affiliation: Department of Ophthalmology, T.N.M.C & B.Y.L Nair Charitable Hospital, Mumbai, India.
authors.forename: Anjali
authors.initials: A
authors.lastname: Nicholson
authors.affiliation: Department of Ophthalmology, T.N.M.C & B.Y.L Nair Charitable Hospital, Mumbai, India.
authors.forename: Anamika
authors.initials: A
authors.lastname: Agrawal
authors.affiliation: Department of Ophthalmology, T.N.M.C & B.Y.L Nair Charitable Hospital, Mumbai, India.
authors.forename: Darshana
authors.initials: D
authors.lastname: Rathod
mesh_terms: D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications* / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D009896:Optic Atrophy / Q000209:etiology*; D010254:Paranasal Sinus Diseases / Q000150:complications / Q000473:pathology*
keywords: CD68; Extra nodal Rosai Dorfman disease; Optic atrophy; S100; Sinus histiocytosis with massive lymphadenopathy