JAAD Case Reports · 2018 · 10 citations · 15 references
MedicineOncologyPathologyCellular PathologyHematopathologyHistopathologyCutaneous LymphomaHematologic PathologyHistiocytic DisordersLymphoid TissueLymphoid Tissue PathologyLymphoid TumorsCutaneous HistiocytosisExtranodal Lymphoid Tissue InvolvementGeneralized LymphadenopathyLocalized LymphadenopathyLymphocytic InfiltratesLymphoma HistopathologyRosai-dorfman DiseaseSinus Histiocytosis
R osai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare, benign histiocytic disorder characterized by generalized lymphadenopathy and constitutional symptoms. The classic presentation of RDD is painless cervical lymphadenopathy with associated fever, night sweats, and weight loss. 2 Extranodal involvement is present in up to 40% of cases of RDD, with the skin being the most common site. 1 Although considered benign, 10% of patients with RDD have coexisting immunologic abnormalities, such as postinfectious conditions and hematologic malignancies. 4]
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