A 13 year-Old Female with Rosai-Dorfman Disease

Rosai-Dorfman or Sinus histiocytosis with massive lymphyadenopathy is a benign self limiting, rare condition of unclear etiology, RDD is of two types familial and other type is associated with infection where titres of Epstein Barr virus and measles virus were found elevated, disturbance in immune system occur in some patients. In some cases human herpes virus 6 DNA has been detected in biopsy samples(1). It may present as massive bilateral cervical lymphadenopathy with systemic symptoms and characterized by histiocytosis and emperipolesis(2,3). RDD is mostly seen in children and young adult population of Caucasians and African origin. In our case a 13 years old female presented in OPD with fever for six months and multiple neck lymph nodes with progressive increase in size over a period of 4 months, one located in the pre-auricular region which was 4 cm , another in the sub-mandibular region approx 3 cm in size and also in sub occipital region 4cm in size and bilaterally palpable lymph nodes along sternocleidomastoid muscle which was more than 6cm in diameter .Clinical examination showed discrete, non-tender, painless, mobile nodes and not attached to skin or underlying structures. There were no signs and symptoms of acute inflammation over the lymph nodes. Biopsy of the nodes was performed and cells expressing positive immunoreactivity for protein S-100 and negative for CD 3 staining which was in keeping with Rosai-Dorfman disease .RDD is a benign pathology, self-limited and various nonspecific therapies available ,the prognosis is excellent