[Pseudotumoral salivary localization of Destombes-Rosai-Dorfman syndrome. Hemophagocytic histiocytosis. Diagnosed by bilateral submandibular and parotid involvement].

We report one case of pseudotumoral salivary location of the Rosai-Dorfman syndrome, with a favorable outcome, occurring in a 32-year-old man from Guadeloupe. The clinical, biological and clinicopathological features of this syndrome, as well as the pathogenetic hypotheses, are summed up. In this case, no bacterial, fungal, parasitic or viral agent was discovered. The subject presented with no immune abnormality likely to account for the persistence and intensity of the histiocytic infiltration with lymphophagocytosis associated to the lymphoplasmocytic infiltration. The rarity of ENT, and more specifically salivary, locations is emphasized. The importance of the detection of possible immune abnormalities in such patients is demonstrated by the outcome of the syndrome being more often unfavorable when such abnormalities are present.