Cutaneous Rosai-Dorfman disease: a systematic review and reappraisal of its treatment and prognosis.

Published: 7 September 2023| Version 1 | DOI: 10.17632/hvyhkng5jd.1


Cutaneous Rosai Dorfman disease (CRDD) is a rare histiocytic disorder that shows distinctive clinical presentation and prognosis. Sufficient data is currently lacking regarding evidence-based management of CRDD. This systematic review aims to provide a comprehensive overview of CRDD, focusing on treatment approaches and outcomes. PubMed and Scopus databases were searched for studies on CRDD from June 1st, 2013 to May 31st, 2023. Articles describing cases of CRDD confirmed with histological examination were eligible for inclusion. All interventions for CRDD were analyzed. The primary outcome measure was the response of cutaneous lesions to treatment including complete response, partial response , and no response. The secondary outcome measures were mortality rate, relapse rate, and the occurrence of adverse events related to CRDD treatment. Eighty-seven articles describing 118 CRDD cases were eligible for this review, the following file describes each article and case individually as it was gathered from each paper on an SPSS spreadsheet.


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PubMed and Scopus databases were searched for eligible studies on May 31st, 2023 using the following MeSH (Medical Subject Headings) items: “Rosai–Dorfman Disease”, “Sinus histiocytosis with massive lymphadenopathy”, “Destombes–Rosai–Dorfman syndrome” combined with “Skin” and “Cutaneous”. Clinical trials, observational studies, case series, and case reports on CRDD published from June 1st, 2013 to May 31st, 2023 were eligible for inclusion. Book chapters, reviews, guidelines, biographies, surveys, quiz papers, and clinical images were excluded. Only papers published in the English language were considered for inclusion. Articles describing cases of CRDD confirmed with histological examination without age restriction were considered for inclusion. We excluded reports of purely nodal RDD and those without cutaneous involvement. Reports that lack detailed data regarding cutaneous involvement of RDD were also excluded. Duplicates were managed with the citation manager software Zotero®. Three authors independently screened titles and abstracts of all articles using the online platform Rayyan®. Then, the same authors independently screened full-text articles for inclusion. Any discrepancies were resolved by consensus. If necessary, a fourth author was consulted to make the final decision. A data extraction sheet was constructed using SPSS® software version 22. Three authors independently performed the initial data extraction for all included articles and a fourth reviewer checked all proceedings. The corresponding authors of eligible articles were asked for additional information if the published data described were insufficient. Extracted data included study design, patients’ demographics, clinical characteristics (age, gender, duration of symptoms, aspects, location, distribution and size of cutaneous lesions, associated symptoms, and systemic involvement i.e., lymph node, hematological, neurological, ocular, ENT, and bone involvement), treatment and outcome.


Universite de Tunis El Manar Faculte de Medecine de Tunis