Rosai-Dorfman Disease : Literature Review

Abstract

 Rosai-Dorfman disease (RDD)—sinus histiocytosis with massive lymphadenopa­thy―represents a peculiar proliferation of histiocyte-like cells in patients. This is a rare benign histiocytic proliferative disorder that was first described by Rosai and Dorfman in 1969, as an idiopathic histiocytic disorder after examining 4 cases.  In 1972, they studied an additional 30 cases of patients with RDD. A histioprolifera­tive disorder, RDD is characterized by bilateral, painless, cervical lymphadenopathy in 81% of patients. Other symptoms such as fever, leukocytosis, elevated sedimentation rate, and polyclonal hypergammaglobu­linemia may also be found. In 30% of patients, extranodal involvement is present and may include the skin, eye orbit, upper respiratory tract, or testes. Cases involving the central nervous system are rare and account for, 5% of patients with RDD. Intracranial disease usually presents clinically and radiologically as a “meningioma”, or as a meningeal process. It can be misdiagnosed as a nonspecific inflammatory process because of the atypical histologic features. Familiarity with the histologic and immunohistochemistry features can afford a definitive diagnosis, and for ruling out a neoplastic process.