S inus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease [RDD]) is a benign, idiopathic, self-limiting lymphoproliferative disease, described by Rosai and Dorfman in 1969. Extranodal manifestations represent about 43% of cases. The disease is classically accompanied by fever, malaise, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. Rosai-Dorfm...

Rosai-Dorfman disease is a rare condition of marrow hematopoietic stem-cell origin. Patients can show extranodal involvement as well as lymphatic involvement, but only about 5% of extranodal cases involve intracranial lesions. A 53-year-old male was admitted to our hospital with bilateral cervical lymphadenopathy. Intracranial tumors and bone lesions were also detected. Cervical lymph node biop...

Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology typically characterized by cervical lymphadenopathy. Extranodal involvement often manifests in the head and neck region. We present a 10-year-old male who presented to our hospital with left epiphora from an aggressive paranasal mass invading the left orbit with osseous destruction. The mass was surgically bi...

UNLABELLED Rosai-Dorfman Syndrome (RDD) is a rare, benign, self-limiting disorder which is characterized by the non-malignant proliferation of distinctive histiocytic cell within lymphatic system. RDD has been described as a dynamic entity in the spectrum of histiocytosis with non-Langerhans cell histiocytosis at one end and Langerhans cell histiocytosis at the other. The exact etiology of this...

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been r...

Rosai-Dorfman disease is a rare histiocyte disorder that is typically characterized by massive cervical lymphadenopathy. Isolated extranodal involvement is uncommon, and isolated laryngeal involvement is extremely rare. We report an unusual case of Rosai-Dorfman disease with isolated laryngeal involvement that led to recurrent dysphonia and airway obstruction. We discuss the challenges we faced...

any patient with bilateral lymphadenopathy especially in indian subcontinent is regarded as suffering from tuberculosis unless proved otherwise. this sometimes leads to unwarranted delay in correct diagnosis and management if there is ignorance regarding other rarer etiologies. rosai-dorfman disease (rdd) may present in the same manner and should always be kept on the back of the mind to help a...

Rosai–Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis that was first described as sinus with massive lymphadenopathy by Rosai and Dorfman in 1969. While classical/nodal RDD typical, extranodal involvement seen up to 43% of all cases, 23% showing only lesions. Here, we present case disseminated RDD, where the initial symptoms were cutaneous manifestations.

BACKGROUND A proportion of cases of Rosai-Dorfman disease exhibit some histological features consistent with IgG4-related disease (IgG4RD). Several investigators have discussed whether Rosai-Dorfman disease belongs to the spectrum of IgG4RD or is concurrent with it by coincidence. OBJECTIVE To elucidate the relationship between the two diseases, we report key features, including IgG4 and amyl...

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare benign idiopathic proliferative disease of phagocytic histiocytes. Approximately 80% of patients present with painless massive cervical lymphadenopathy. Isolated extranodal involvement is relatively uncommon. Two cases of Rosai-Dorfman disease are reported: one with isolated bilateral orbital involvement and one ...