Rosai-Dorfman-Destombes (RDD) disease presenting as palindromic rheumatism

Amin Haghighat Jahromi, Aaron M. Goodman, Carl K. Hoh

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Background: Rosai-Dorfman-Destombes (RDD) disease, is a rare proliferative and inflammatory disorder of non-Langerhans cell histiocytes. Case presentation: We report a 35-year-old woman, who originally presented with recurrent episodes of lower extremity joint/bone pain and chronic nasal stuffiness and congestion. Her worsening nasal congestion was due to an obstructing nasal cavity lesion which was subsequently biopsied. Pathology was consistent with RDD. 18F-FDG PET images demonstrated intense uptake in the paranasal sinuses and a large pelvic lymph node. Focal osseous lesions with intense 18F-FDG uptake were also observed in the lower extremity, corresponding to areas of peri-articular pain. Rheumatologic work-up was consistent with palindromic rheumatism. She was diagnosed with immune-related disseminated RDD, presenting as palindromic rheumatism. Conclusions: This is the first case of RDD presenting as palindromic rheumatism. RDD should be considered as a possible but rare diagnosis in young patients with sinus-related symptoms and lymphadenopathy. The disease can on rare occasions be disseminated and can also present as immune-related RDD, such as in this patient.

Original languageEnglish
Article number72
JournalBMC Medical Imaging
Volume21
Issue number1
DOIs
StatePublished - Dec 2021

Keywords

  • F-FDG PET/CT
  • Histiocytosis
  • Palindromic rheumatism
  • Rosai-Dorfman-Destombes disease
  • Sinus histiocytosis with massive lymphadenopathy

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