Enfermedad de Rosai-Dorfman con afección ganglionar y extraganglionar

Abstract

Introduction: Rosai-Dorfman disease, known as sinus histiocytosis with massive lymphadenopathy, is a very rare variety of reactive histiocytosis of unknown origin. Case presentation: a 32-year-old female patient who consulted for a five-year history of respiratory distress, dysphonia, stridor and multiple swollen areas in the neck and trunk. Physical examination revealed large, firm, and mobile submandibular lymph nodes and numerous nodules on the trunk. Histological examination showed the characteristic features of Rosai-Dorfman disease with nodal and extranodal involvement. Discussion: Rosai-Dorfman disease is a pathological process which affects cervical lymph nodes, although lesions may develop in several sites, organs, and systems, and may be confused with other neoplasms. It should be considered in the differential diagnosis of soft tissue histiocytic lesions and other lymphoproliferative disorders. Death is related to organs and systems dysfunction rather than to the disease itself. Conclusion: Rosai-Dorfman disease including nodal and extranodal involvement, is a very rare benign proliferative histiocytic disorder. It should always be included in the differential diagnosis of other histiocytic proliferative disorders. It carries a variable relatively benign prognosis.