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Acta Médica Costarricense

On-line version ISSN 0001-6002Print version ISSN 0001-6012


PORRAS-MORENO, Adriana  and  VALVERDE-MUNOZ, Kathia. Unusual disease in pediatrics: Rosai Dorfman disease. Acta méd. costarric [online]. 2018, vol.60, n.1, pp.42-44. ISSN 0001-6002.

Rosai Dorfman’s disease is a rare entity characterized by non-painful lymphadenopathies predominantly cervical and submandibular. In the past it had been considered a malignant reticuloendotheliosis, until it was described as a clinical-pathological entity, isolated in 1969 by Rosai and Dorfman. We present the case of a 4-year-old girl, known to be healthy, with an acute condition of febrile sensation, cervical mass and leukocytosis. Laboratory studies did not identify malignancy or infection as an etiology. She was treated with antibiotics without resolution of his adenopathy, which led to an excisional biopsy. The histological study revealed a sinus histiocytosis with massive lymphadenopathy. It was decided to give conservative management since it had a complete resection.

Keywords : Rosai-Dorfman; Sinus histiocytosis; lymph nodes.

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