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Rosai-Dorfman-Destombes (RDD) disease presenting as palindromic rheumatism
Amin Haghighat Jahromi1, Aaron M Goodman2, Carl K Hoh3
1Department of Radiology, University of California, UCSD Medical Center, 200 W Arbor Drive, San Diego, CA, 92103-2686, USA. ajahromi@ucsd.edu.
Rosai-Dorfman-Destombes (RDD) disease, a rare histiocytic disorder, can present unusually. This case highlights RDD manifesting as palindromic rheumatism in a young woman with sinus symptoms.
Area of Science:
- Histiocytic Disorders
- Immunology
- Rheumatology
Background:
- Rosai-Dorfman-Destombes (RDD) disease is a rare, non-Langerhans cell histiocyte proliferative and inflammatory disorder.
- Disseminated RDD is uncommon but can present with diverse symptoms.
Observation:
- A 35-year-old woman presented with joint pain, nasal congestion, and sinus lesions.
- Imaging revealed intense 18F-FDG uptake in paranasal sinuses, pelvic lymph nodes, and lower extremity osseous lesions.
- Rheumatologic work-up indicated palindromic rheumatism.
Findings:
- Histopathology confirmed Rosai-Dorfman-Destombes (RDD) disease.
- The patient was diagnosed with immune-related disseminated RDD presenting as palindromic rheumatism.
- This represents the first reported case of RDD presenting with palindromic rheumatism.
Implications:
- RDD should be considered in young patients with sinus symptoms and lymphadenopathy.
- The potential for disseminated and immune-related presentations of RDD is highlighted.
- This case broadens the clinical spectrum of Rosai-Dorfman-Destombes (RDD) disease.
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