[Destombes-Rosai-Dorfman syndrome: 2 uncommon clinical forms]

Two cases of Destombes Rosai Dorfman's syndrome are presented. Diagnosis was performed by superficial lymph node biopsy. The first case concerned a nine and half years old girl with cervical adenopathy who developed a compressive mediastinal adenopathy responsible for a right lower lobe atelectasis. Because of local lung suppuration a lobectomy had to be performed. The second case concerned a fourteen years old boy with recurrent fever, diffuse superficial lymph nodes and erythematous skin rash. The two patients showed clinical and biological inflammatory symptoms without any immunodeficiency. No aetiological agent could be identified. Antibiotics and corticoids had no effect but the two patients recovered (after 18 months follow up in case 2). These two particular cases confirm the clinical course heterogeneicity of the syndrome which requires histological diagnosis.