Cutaneous Rosai-Dorfman Disease of the Face: A Comprehensive Literature Review and Case Report

Abstract

Purpose: Cutaneous Rosai-Dorfman disease (C-RDD) is a rare, benign, non-Langerhans cell histiocytosis that can affect any skin area. The purpose of this study was to characterize C-RDD of the face through a literature review of published case reports of this lesion and present a new case of C-RDD.

Materials and methods: An English-language search of 3 databases (PubMed, Scopus, and EBSCO Search) was conducted for cases of C-RDD of any skin area that had been published since 1969. Repeated citations of the same article in more than 1 database were deleted. Cases of C-RDD with solely facial involvement or involving the face and other skin parts were included and analyzed. Details of C-RDD cases (demographic features, regional distribution, clinical presentation, treatment, and follow-up course) were methodically reviewed and collected in Excel spreadsheets. Simple statistical analyses were conducted using Excel.

Results: The literature search yielded 578 published cases of C-RDD affecting any skin area; of these, 65 cases (11.2%) had facial skin involvement. The male-to-female ratio was 1:1.5, and the average age at presentation was 43.5 years (standard deviation, 12.4 yr). The racial distribution of facial C-RDD was, in descending order, 74.5% in Asians, 20% in Caucasians, and 5.5% in blacks. The most commonly affected facial skin regions were the cheeks and periorbital area, and most lesions were multiple in number and bilaterally distributed. The vast majority of facial C-RDD lesions presented as asymptomatic, nonulcerative, red, nodular plaques with durations ranging from 1 month to a few years. Many methods have been attempted for the treatment of facial C-RDD. However, the combined cure rate for all published treatment methods was only 28.6%. Surgical excision was the most effective treatment method, and corticosteroids were the least effective.

Conclusion: This article has tried to characterize facial C-RDD lesions for easier management by maxillofacial surgeons.