Rosai Dorfman Disease: A Case Presentation

Abstract

Rosai Dorfman Disease (RDD), also called Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare proliferation of histiocytes, of unknown cause.¹ The term was first used in 1969, by Rosai and Dorfman.^2,3 It is a massive bilateral adenopathy of head and neck nodes with fever, raised Erythrocyte sedimentation rate (ESR) and hypergammaglobulinaemia, usually in young patients in first 2 decades of life.^3,4,5Extranodal involvement is reported in 10%- 40% of patients, with involvement of respiratory tract, genitourinary tract, nasopharynx, paranasal sinuses, gastrointestinal tract, orbit and the eye. ^6,7 The site of occurrence determines the clinical features like nasal obstruction, stridor, proptosis and ptosis.⁸ Isolated orbital involvement without systemic clinical features is uncommon.⁹ Patients with ophthalmic manifestations are younger with poorer visual acuities and patients with ocular involvement have multiorgan disease, which is more limited in those with orbital masses.10 Involvement of the orbit, eyelid and cornea have been reported.^11,12 Ocular involvement may present as cervical lymphadenopathy with poorly responsive anterior uveitis.13 Aetiologic theories include immune regulatory disorder and viruses such as Herpes and Epstein-Barr.14 Association with sickle cell disease is documented and an African Caribbean black predilection has been found.^15,16 Histology demonstrates phagocytized, intact lymphocytes and erythrocytes, engulfed within intact histiocytes, termed Emperipolesis.^2,3,9 Immunohistochemical stains are positive for S- RDD may be self-limiting, with spontaneous remission in over 50% of patients, or with a relapsing and remitting pattern.¹⁷ Treatment include observation, excision, systemic steroids, chemotherapy and radiation.^6,18,19