The Destombes-Rosai-Dorfman Disease or Sinus Histiocytosis with Massive Lymphadenopathy: A Rare Case Described in the Paediatrics Department of Yalgado Ouedraogo University Hospital
Destombes-Rosai-Dorfman’
disease (DRD) is an inflammatory non-langerhansian histiocytosis with adenomegaly. It is a rare histiocytic disorder worldwide,
less than 1000 reported cases. It is of unknown etiology and is characterized
in its classical form by multiple adenomegalia, especially localized at
cervical area, but also at mediastinal,
axillary and inguinal areas. It is a benign condition,
even if it is
deforming, with spontaneously resolving evolution. We report a case of DRD disease in a 13-year-old girl,
hospitalized in the Department of Pediatrics ofthe Yalgado Ouédraogo
University Hospital Center in Ouagadougou, in March 2015. She was
admitted for a voluminous bilateral painless cervical swelling, with no sign of
local compression, having been operating for about a year. Biological tests
showed signs of chronic inflammation; the imagery specified the benign
character of these formations: adenomegalia. Confirmation of the diagnosis was
made by pathological examination, describing the aspect of emperipolesis to
histology and, immunohistochemistry, polytypic plasmacytosis and PS100
positive/CD1a histiocytosis negative. The treatment could not be properly carried out, in view of the early
discharge, against medical advice from the patient. The evolution would
have been marked, according to the parents, by a slight involution of swelling.
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