Rosai-Dorfman Disease: Self-Resolving UnilateralLymphadenopathy and a Brief Review of Literature

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a disease of histiocyticproliferation with no known pathogenesis. This disease is defined by histological and cytological characteristics, includingemperipolesis and positive S100 and CD68 markers. Although the disease typically presents clinically with massive bilaterallymphadenopathy due to sinus expansion from excessive histiocytosis, only unilateral lymphadenopathy was observed in thispatient. The case involves a 40-year-old Caucasian male from the Netherlands presenting with unilateral lymphadenopathysuspicious for malignancy. Subsequent histological and laboratory testing led to the rare diagnosis of RDD. The lymphadenopathyresolved spontaneously over the course of several weeks following the initial presentation. Rosai-Dorfman disease reportedly has abenign prognosis with approximately 20% of patients experiencing spontaneous disease resolution (as was the case for thispatient) with 70% experiencing chronic symptoms that may last years but not require intervention. We therefore advocateobservation as a mainstay of treatment for most cases of this rare disease with intervention only being pursued in symptomaticcases. A review of recent literature regarding pathogenesis, epidemiology, diagnostic factors, prognosis, and treatment is providedand discussed.