Two cases of Destombes Rosai Dorfman's syndrome are presented. Diagnos
is was performed by superficial lymph node biopsy. The first case conc
erned a nine and half years old girl with cervical adenopathy who deve
loped a compressive mediastinal adenopathy responsible for a right low
er lobe atelectasis. Because of local lung suppuration a lobectomy had
to be performed The second case concerned a fourteen years old boy wi
th recurrent fever, diffuse superficial lymph nodes and erythematous s
kin 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 pati
ents recovered (after 18 months follow up in case 2). These two partic
ular cases confirm the clinical course heterogeneicity of the syndrome
which requires histological diagnosis.