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Background: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant disorder characterized by excessive proliferation of histiocytes, the cause of which remains unknown. Although the lymph nodes are the most commonly affected site, some patients may present with extranodal involvement, particularly in the skin, nasal cavity, eyes, and bones. In this report, we aim to present a unique case of RDD with pleural involvement in a 61-year-old patient.

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In the present case, a 66-year-old woman presented to the Specialty Hospital (Amman, Jordan) with recurrent post-menopausal bleeding. A pelvic ultrasound scan showed an abnormal endometrial thickness of 8 mm and no adnexal masses. An endometrial biopsy revealed abundant foamy histiocyte infiltration features suggestive of xanthogranulomatous endometritis.

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Introduction: The aim of this study was to describe the clinical features and treatment responses of Rosai-Dorfman disease (RDD) and Erdheim-Chester disease (ECD) with kidney involvement.

Methods: We retrospectively analyzed patients with RDD and ECD with kidney involvement from 2005 to 2023, evaluating kidney function changes, as well as computed tomography (CT), and metabolic responses.

Results: The study included 4 patients with RDD and 44 with ECD, with median ages of 58 and 51 years, respectively.

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Rosai-Dorfman disease (RDD) is a rare proliferative disorder of histiocytes, and primary solitary RDD of the bone is extremely rare. Some RDDs exhibit increased immunoglobulin (Ig)G4 positive (IgG4+) plasma cell infiltration and the histopathological features of IgG4-related disease (IgG4-RD). However, the association between RDD and IgG4-RD remains unclear.

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Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children.

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