Sinus Histiocytosis (Rosai Dorfman Syndrome)
Discussion: Sinus histiocytosis with massive lymphadenopathy is a self limited syndrome. Patients typically present with bilateral non-tender cervical lymphadenopathy (97%) and fevers. Extra nodal disease is seen in 30% of patients including cutaneous, trachobronchial and fatty infiltration. Sinus histiocytosis is part of a larger syndrome which includes all the histiocytosis infiltrating processes. Although most are self limited and resolve (50%), there is persistent lymphadenopathy and symptomatology in 15%.
References:
Silvers and Som. AJNR February 1996.