Progress in the diagnosis and treatment of Rosai-Dorfman disease

Abstract

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis. Its etiology and pathogenesis are yet incompletely elucidated. The clinical manifestations of RDD are highly heterogeneous. It is easy to be misdiagnosed or missed diagnosis. There is no standard treatment at present. Therefore, the progression in pathogenesis, diagnosis and treatment are reviewed. The treatment of RDD is best tailored to the individual clinical circumstances. Observation is suitable for patients with uncomplicated lymphadenopathy or asymptomatic cutaneous RDD. For RDD patients with extra nodal unifocal disease or organ compression, surgery is recommended. Systemic therapy can be considered for patients with systemic, recurrent or refractory RDD.

Keywords

• rosai-dorfman disease|clinical manifestations|treatment