Synonyms: Destombes-Rosaï-Dorfman disease | Rosaï-Dorfman-Destombes disease | SHML | Sinus histiocytosis with massive lymphadenopathy
A rare non-Langerhans cell histiocytosis characterized by infiltration of lymph nodes or extranodal tissues by non-malignant histiocytes displaying emperipolesis a non-destructive phagocytosis of lymphocytes or erythrocytes. Most typical presentation is as a massive cervical lymphadenopathy in adolescents and young adults. Most frequent sites of extranodal disease are skin soft tissue bones paranasal sinuses orbit salivary glands and central nervous system. Symptoms are related to mass effect in the affected organs.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2026
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Rosaï-Dorfman disease?
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Advocacy Organizations
The Dynamite Foundation Inc
The Dynamite Foundation is on a heartfelt mission: to bring love, hope, and joy to individuals impacted by Histiocytosis around the world We do this through Dynamite the Histio Warrior Dragon™; a handcrafted crochet dragon sent to Histio Warriors in the midst of their battles, and to Angel Families remembering their loved ones through Angel Dynamite.
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Clinical Trials
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