[Two cases of sinus histiocytosis with massive cervical lymphadenopathy (Destombes-Rosai-Dorfman disease)]

Abstract

Introduction: Sinus histiocytosis with massive lymphadenopathy is a rare disease. Its location is ubiquitous, but the most frequent is cervical lymph nodes.

Observation: We report 2 cases of sinus histiocytosis with massive lymphadenopathy. The patients were respectively 9 and 30 years of age. They both presented with bilateral cervical polylymphadenopathy with a cervical compression syndrome in the first patient. The diagnosis was made histologically by demonstrating histiocyte emperipolesis, in the first case, and by immuno-histochemistry in the second. The treatment was corticosteroids, resulting in an almost total involution.

Discussion: Sinus histiocytosis with massive lymphadenopathy is a clinical and a specific pathology, but its etiopathogenesis remains to be elucidated. The cervical localization is common. The definitive diagnosis is histological. There is currently no codified treatment. The outcome is usually favorable, apart from mechanical and systemic complications that are important prognostic factors.

Keywords: Destombes-Rosai-Dorfman disease; Histiocytose sinusale; Histiocytosis; Lymphadénopathie massive; Maladie de Destombes-Rosai-Dorfman; Massive lymphadenopathy; Sinus.