[Haemophagocytic sinus histiocytosis or Destombes-Rosai-Dorfman syndrome (author's transl)].
Haemophagocytic sinus histiocytosis usually presents as cervical lymphadenopathy developing in a context of severe inflammatory reaction. The lymph nodes contain numerous large cytophagic histiocytes capable of absorbing all kinds of blood cells, and diffuse plasmocytic infiltrates. Other organs may be involved, notably the skin and bones, as in the case presented here. The course of the disease if prolonged, irrespective of the treatment administered.
D001847:Bone Diseases / Q000209:etiology; D006801:Humans; D008206:Lymphatic Diseases / Q000473:pathology*; D008297:Male; D012877:Skin Manifestations
Histiocytose sinusale hémophagocytaire ou syndrome de Destombes-Rosai-Dorfman.