Dysphagia in the setting of Rosai–Dorfman disease

A 72-year-old female presented with 3 weeks of difficulty swallowing to both solids and liquids. She had associated nausea, weight loss of 12 lbs, and night sweats. There was a suspicion of esophageal malignancy. She underwent EGD showing narrowing of the esophagus. To further evaluate the narrowing she underwent CT with IV contrast showing lymphadenopathy in the mediastinum and mesentery. This further led to evaluation for lymphadenopathy including HIV, fungitell, LDH, peripheral smear, Bartonella serology, and Quantiferon. Labwork was largely unremarkable. Finally, she underwent EBUS guided node biopsy which showed sinus histiocytosis or Rosai–Dorfman disease. She was started on steroid taper with Prednisone at 1 mg/kg with a 6-week taper. This led to a drastic improvement in night sweats and dysphagia. The buildup of aberrant cells, or histiocytes, in different human tissues and organs is a characteristic of RDD. RDD can affect any organ system from head to toe, however it typically affects the skin and lymph nodes. Though various theories, such as bacterial, viral, or environmental sources, have been proposed, the exact cause of these cells' overproduction remains unknown. While RDD does occur globally and appears to affect an equal number of males and females, the precise incidence of cases is unknown. Both adults and children may be impacted by it. Due to the extreme rarity of this illness, no significant research has been done, and there is currently no recognized cure.

Publication History

Article published online:
27 March 2025