Rosai Dorfman disease: a rare entity
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Date
2025-07
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Publisher
Medip Academy
Abstract
Rosai Dorfman disease (RDD), a rare benign proliferative disorder of unknown etiology, stems from abnormal histiocyte accumulation within lymph node sinusoids and extra nodal tissues. While uncommon in paediatric population, RDD clinically simulates lymphoproliferative disorders. This report details a 4-year male patient presenting at a tertiary care centre with progressive bilateral cervical and submandibular lymphadenopathy lacking febrile symptoms. Diagnostic confirmation was achieved through excisional biopsy. RDD is an exclusion-based diagnosis which requires multidisciplinary collaboration and heightened clinical suspicion remains essential for paediatricians and oncologists to facilitate timely intervention and management of this rare entity.
Description
Keywords
Lymph nodes, Histopathology, Non-Langerhan cells, Steroids
Citation
Goyal S, Sharma CM, Garg C. Rosai Dorfman disease: a rare entity. International Journal of Contemporary Pediatrics. 2025 Jul; 12(7): 1226-1228