Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): two case reports

Sinus histiocytosis with massive lymphadenopathy or Destombes-Rosai-Dorfman 's syndrome is a rare benign disease of unknown etiology. usually seen in y ounger patients, The cases reported concerned a 15-month old Caucasian boy and an 8 year old black boy with unilateral cervical enlargement, occasiona l fever and without any extranodal involvement. Diagnosis was performed by superficial lymph node biopsy. No immunodeficiency was found. The patients received no therapy and a complete spontaneous resolution was seen after a few months in the two cases. The clinical presentation, histologic characte ristics, pathogenesis and treatment of the Destombes-Rosai-Dorfman's syndro me are discussed. (C) 2001 Elsevier Science Ireland Ltd. All rights reserve d.