Orphanet: Rosaï Dorfman disease

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Rosaï-Dorfman disease

Disease definition

Rosaï-Dorfman disease is a rare benign non-Langerhans cell histiocytosis characterized by the development of large painless histiocytic masses in the lymph nodes, predominantly of the cervical region. Extranodal involvement can also be observed, such as in the skin, respiratory tract, bones, genitourinary system, soft tissues, oral cavity, and central nervous system. Additional findings may include fever, malaise, epistaxis, night sweats, weight loss, leukocytosis, elevated erythrocyte sedimentation rate and hypergammaglobulinemia.


Classification level: Disorder
  • Synonym(s):
    • Destombes-Rosaï-Dorfman disease
    • Rosaï-Dorfman-Destombes disease
    • SHML
    • Sinus histiocytosis with massive lymphadenopathy
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Adolescent, Adult
  • ICD-10: D76.3
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: 7588
  • MedDRA: 10063397

Detailed information


The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.