Le syndrome de destombes-rosai-dorfman [a propos d'un cas]

ABSTRACT

The sinus histiocytosis with massive lymphadenopathy or Destombes-Rosai-Dorfman syndrome is a reactional nodal histiocytosis which is mainly found in children and adolescents, characterised by bilateral and voluminous lymphadenopathy with a proconsular neck. We report a tunisian case of 10 years-old male who presented with general health deterioration and developed later voluminous bilateral, cervical lymphadenopathy. A nodal biopsy permitted the diagnosis of massive lymphadenopathy of Destombes-Rosai-Dorfman. The response to corticotherapy was favorable with improvement of general health and regression of the lymphadenopathy