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

Case Reports

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

Amin Haghighat Jahromi et al. BMC Med Imaging. 2021.

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.

Keywords: 18F-FDG PET/CT; Histiocytosis; Palindromic rheumatism; Rosai-Dorfman-Destombes disease; Sinus histiocytosis with massive lymphadenopathy.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1

Fig. 1

Contrast-enhanced maxillofacial MRI demonstrates left ethmoid sinus lesion. a Enhancing infiltrative lesion in the left anterior ethmoidal sinus in the Gadolinium-enhanced sequence (White arrow). b This lesion is hypointense in the T2-weighted sequence. c, d The lesion is hypointense in the apparent diffusion coefficient (ADC) sequence and mildly hyperintense in the diffusion-weighted imaging (DWI) sequence, likely due to hypercellularity

Fig. 2

Fig. 2

Whole-body 18F-FDG PET co-registered with contrast-enhanced maxillofacial MRI demonstrates sinus lesions. a Whole-body FDG PET maximum intensity projection image demonstrates intense uptake in the nasal cavity, paranasal sinuses, left pelvis, left knee and left ankle (black arrows). Nonspecific mild uptake in the right ankle, and left side of the neck are also seen. b Mildly enhancing infiltrative lesion in the left anterior ethmoidal sinus in the Gadolinium-enhanced maxillofacial MRI, demonstrates intense FDG uptake in the fused PET/MRI axial image (SUVmax 10.6, white arrow). c Enhancing infiltrative mass in the left middle nasal turbinate, in the Gadolinium-enhanced maxillofacial MRI, demonstrates intense FDG uptake in the fused PET/MRI axial image (SUVmax 7.7, white arrow)

Fig. 3

Fig. 3

Whole-body 18F-FDG PET/CT demonstrates pelvic lymphadenopathy and osseous lesions. a Intense uptake in a 3.8 × 3.6 cm left pelvic side wall lymph node on the fused PET/CT axial image (SUVmax 9.8, white arrow). b Moderate FDG uptake in the left lateral femoral condyle (SUVmax 3.1, white arrow), on the fused PET/CT axial image, correlating with the mixed lytic/sclerotic subchondral lesion in the CT. c Intense FDG uptake in the left talus on the fused PET/CT axial image (SUVmax 7.7, white arrow) correlating with mixed lytic/sclerotic subchondral lesion on the CT

References

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