Disseminated Rosai-Dorfman Disease with Multiple Extranodal Involvement: A Rare Case Report

Abstract

Abstract:
Sinus histocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is an uncommon benign tumor characterized by idiopathic proliferation of phagocytic histiocytes. RDD is usually manifested by the presence of large painless lymphadenopathy; however, extranodal involvement, e.g., the central nervous
system, orbits, skin and respiratory tract, is rarely seen. Herein, we reported an elderly man presenting a 3-month history of progressive generalized papules with lymphadenopathies. The pathological findings of the skin, cervical lymph node and bronchial nodule were consistent with RDD. He received a diagnosis of disseminated
RDD. Treatment with immunosuppressive therapy using corticosteroid and mercaptopurine resulted in gradually improved symptoms within 3 weeks.