Abstract
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 of the Yalgado Ouedraogo 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.
Highlights
Destombes-Rosai-Dorfman’ s disease (DRD) is an inflammatory non-langerhansian histiocytosis with adenomegaly [1], described by Destombes in 1965 and later, better defined by Rosai and Dorfman in 1969 [2].It is a clinicopathological entity rarely described, infant-juvenile (10 - 20 years), of chronic evolution; less than 1000 cases listed around the world according to the histiocyte society [3]
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, and at mediastinal, axillary and inguinal areas
We report a case of DRD disease in a 13-year-old girl, hospitalized in the Department of Pediatrics of the Yalgado Ouédraogo University Hospital Center in Ouagadougou, in March 2015
Summary
Destombes-Rosai-Dorfman’ s disease (DRD) is an inflammatory non-langerhansian histiocytosis with adenomegaly [1], described by Destombes in 1965 and later, better defined by Rosai and Dorfman in 1969 [2]. It is a clinicopathological entity rarely described, infant-juvenile (10 - 20 years), of chronic evolution; less than 1000 cases listed around the world according to the histiocyte society [3].
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