Rosai Dorfman disease- A rare entity: Case report in North Indian male

Abstract

Rosai Dorfman disease is a rare disease characterized by histiocytic proliferation of lymph node in young adults. We present here the case of a 67 years old Indian male presenting with lymphadenopathy and pyrexia of unknown origin for 16 months duration. Histopathological examination of lymph nodes revealed emperipolesis consistent with the diagnosis of Rosai Dorfman disease. The patient recovered with steroid treatment.Our case is one of the few cases of adult onset Rosai Dorfman Disease reported from India.

Rosai-Dorfman disease of the trachea: an extremely rare benign tumor

“…Histological hallmarks of the disease include emperipolesis, positive immunohistochemical staining with S100 protein and CD68, and negative staining with CD1a. 1,2,6,7 Our case was S100-positive. Management of tracheobronchial RDD depends on the presentation.…”

“…Rosai-Dorfman disease was classified as a distinct clinicopathological disorder in 1969. 2 The typical presentation consists of fever, leukocytosis, and painless cervical lymphadenopathy in 87% of cases, while other lymph node involvement has been reported in 43% of cases. 1 Intrathoracic involvement is extremely rare, reported in 2% of cases, including pulmonary nodules, pleural effusion, or airway involvement.…”

“…Radiotherapy has been recommended by a few authors, but conclusive guidelines are lacking. 2 Recurrence has been reported in several cases, 1,5 but long-term studies are lacking.…”