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

BMC Medical Imaging

Aaron M. Goodman¹  Carl K. Hoh²  Amin Haghighat Jahromi² 

[1] Department of Medicine, Division of Blood and Marrow Transplantation, Moores Cancer Center, University of California, San Diego, CA, USA;Department of Radiology, University of California, UCSD Medical Center, 200 W Arbor Drive, 92103-2686, San Diego, CA, USA;

关键词: Sinus histiocytosis with massive lymphadenopathy;    Rosai-Dorfman-Destombes disease;    Histiocytosis;    Palindromic rheumatism;    F-FDG PET/CT;   

DOI  :  10.1186/s12880-021-00596-2

来源: Springer

【 摘 要 】

BackgroundRosai-Dorfman-Destombes (RDD) disease, is a rare proliferative and inflammatory disorder of non-Langerhans cell histiocytes.Case presentationWe 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.ConclusionsThis 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.

【 授权许可】

CC BY