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Rosai-Dorfman disease: A Review of Recent Literature (2019-2026)
Rosai-Dorfman Disease (RDD),also known as histiocytic granuloma,is a rare,non-Langerhans cell histiocytosis characterized by massive lymphadenopathy,often accompanied by systemic symptoms. While classically presenting with cervical lymph node involvement,cutaneous RDD (cRDD) represents a distinct,though less common,presentation. This review summarizes recent literature (2019-2026) regarding the diagnosis, clinical presentation, and management of RDD, with a focus on cutaneous manifestations.
Clinical Presentation & Diagnostic Challenges:
Recent case reports (Amir et al., 2024; touati et al., 2024) highlight the diagnostic challenges posed by cRDD, often mimicking other dermatological conditions. Clinically, cRDD can present as solitary or multiple papules, nodules, or plaques, sometimes resembling basal cell carcinoma (Amir et al., 2024) or lipomas (Touati et al.,2024). The face is a common site of involvement.A key diagnostic feature is the presence of large histiocytes with emperipolesis – the engulfment of lymphocytes by histiocytes – on histopathological examination.
However, differentiating RDD from other histiocytic disorders and IgG4-related disease can be difficult. Zghal et al. (2022) reported a case of primary cutaneous RDD with features mimicking IgG4-related disease, emphasizing the importance of thorough immunohistochemical staining. Specifically, RDD is typically positive for S-100 and CD68, while negative for IgG4.
The identification of MAP2K1 mutations is increasingly recognized as critically important in the diagnosis of RDD,particularly in cases with atypical presentations (Gillam et al., 2024). this mutation is found in a significant proportion of RDD cases and can aid in distinguishing it from other histiocytic proliferations.
Systemic involvement & Differential Diagnosis:
While cRDD is often localized, it can be associated with systemic disease. Deen et al. (2022) provide a review of diagnostic testing and management of extranodal RDD, highlighting the need to evaluate for involvement of other organs. the differential diagnosis includes other non-Langerhans cell histiocytoses, infections (Hay et al., 2019), and IgG4-related disease. A thorough workup, including imaging studies and laboratory tests, is crucial.
Treatment & Prognosis:
The treatment of RDD remains largely supportive, as the disease frequently enough follows a self-limiting course. however,cases with significant systemic involvement or persistent cutaneous lesions may require intervention. Dhrif et al. (2024) conducted a systematic review and reappraisal of treatment and prognosis, finding that treatment options range from observation to systemic therapies such as corticosteroids, chemotherapy, and targeted therapies (e.g., MEK inhibitors for MAP2K1-mutated cases).
Mohaghegh et al. (2025) reported on two cases and reviewed recent literature, noting that the prognosis of cRDD is generally favorable, but long-term follow-up is recommended to monitor for recurrence or systemic involvement. Rarely, RDD can be associated with the development of hematologic malignancies, as reported in a case by Amir et al. (2024) where multiple myeloma developed two years after the initial diagnosis of cRDD.
Conclusion:
Cutaneous rosai-Dorfman disease is a rare but important entity that clinicians should consider in the differential diagnosis of unusual skin lesions