Introduction And Importance: Rosai-Dorfman disease (RDD) is a rare disorder characterized by benign histiocytic proliferation. The purely cutaneous variant of Rosai-Dorfman disease is exceptionally uncommon. This abstract centers on an extraordinary case-an instance of primary cutaneous Rosai-Dorfman disease (PCRDD), a rare cutaneous variant within an already infrequent disorder. Successfully managed through surgical intervention, this unique case underscores the pressing need for refined diagnostic and therapeutic strategies.
Case Report: We present a noteworthy case of primary cutaneous Rosai-Dorfman disease (PCRDD) in a north African female, deviating from the typical age of onset. The patient exhibited a rare cutaneous variant, emphasizing the need for heightened awareness in diverse demographics. Histological analysis revealed characteristic emperipolesis and immunohistochemical features consistent with RDD.
Clinical Discussion: PCRDD, although uncommon within the Rosai-Dorfman Disease spectrum, manifests with a later onset and distinctive demographic characteristics. Surgical excision emerges as a viable therapeutic option for managing symptomatic lesions, as demonstrated in our case. The infrequency of PCRDD underscores the importance of tailored diagnostic and therapeutic strategies. Individualized approaches become pivotal in addressing the complexities associated with PCRDD, ensuring careful consideration of its intricacies in the broader context of Rosai-Dorfman disease.
Conclusion: This case underscores the complexity of RDD, especially in rare cutaneous presentations. Surgical excision, with its reported efficacy, stands as a crucial intervention, offering both symptomatic relief and potential cure, showcasing the significance of individualized care in managing this rare disorder. Continued research remains imperative for advancing RDD understanding and refining clinical approaches.
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http://dx.doi.org/10.1016/j.ijscr.2024.109475 | DOI Listing |
Ann Thorac Surg Short Rep
September 2024
Division of Cardiac Surgery, University of British Columbia, Vancouver, British Columbia, Canada.
Rosai-Dorfman disease (RDD) is a nonmalignant disease of histiocyte proliferation. RDD usually presents with painless cervical lymphadenopathy, although extranodal involvement can occur. Cardiac involvement was reported in <0.
View Article and Find Full Text PDFEur J Breast Health
January 2025
Department of Radiology, Miller School of Medicine, University of Miami, Miami, FL, USA.
Rosai-Dorfman disease (RDD) is a self-limited, idiopathic, non-neoplastic disorder characterized by the proliferation of phagocytic histiocytes, which can mimic malignant lymphoproliferative disease. Cases of RDD most commonly present as bilateral painless cervical lymphadenopathy, with lesser involvement of the axilla, inguinal, and mediastinal lymph nodes. We present the case of a 62-year-old woman with a history of endometrial serous carcinoma who underwent evaluation at a dedicated breast imaging department after positron emission tomography/computed tomography (PET/CT) revealed breast masses and axillary nodes with increased uptake of fluorodeoxyglucose (FDG).
View Article and Find Full Text PDFCureus
November 2024
Hematology and Medical Oncology, Kettering Health, Kettering, USA.
Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder characterized by sinus histiocytosis with massive lymphadenopathy, rarely presenting with severe and life-threatening extra-nodal features. The rarity of RDD, clinically variant phenotype, limited data, and lack of a current standardized management approach make treatment decisions difficult. Herein, we present a case of life-threatening, disseminated RDD with rare clinical features of recurrent pericardial effusion, bilateral pleural effusions, and abdominal tissue fibrosis successfully treated with six cycles of cladribine, achieving clinical remission.
View Article and Find Full Text PDFInt J Surg Case Rep
December 2024
Department of Surgical Oncology, Regional Hospital of Jendouba, Jendouba, Tunisia.
Introduction: Rosai-Dorfman disease (RDD) is a rare histiocytic neoplasm. It most commonly presents with bilateral cervical lymphadenopathy. We report the fourth case in the literature of RDD presenting as isolated axillary lymphadenopathy.
View Article and Find Full Text PDFArch Dermatol Res
December 2024
Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia.
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