Pure Cutaneous Rosai Dorfman Disease: An Uncommon Location for a Rare Histiocytic Proliferative Disorder.

Content Provider	Europe PMC
Author	Jain, Manjula Pujani, Mukta Sidam, Dipti Wadhwa, Ruchira Passi, Shanta Uikey, Deepika
Copyright Year	2021
Abstract	RosaiDorfman disease (RDD) is a rare, self-limiting disease of uncertain etiology involving lymph nodes as well as extranodal sites. Isolated or pure cutaneous RDD (PCRDD) without lymph node involvement is very unusual accounting for only 3% of all described cases of RDD. The clinical features of PCRDD are quite different from RDD according to the literature on extranodal RDD, thereby emphasizing that PCRDD is a distinct clinical entity. Histopathology remains the gold standard for the diagnosis of both systemic and PCRDD with the presence of characteristic emperipolesis observed in histiocytes while immunohistochemistry (IHC) (S100, CD 68 positive, and CD 1a-negative) serves as a useful adjunct. We hereby report a case of a 36-year-old female who presented with a tender, indurated plaque on the left forearm with a clinical differential diagnosis of borderline tuberculoid leprosy/sarcoidosis and lupus vulgaris. However, on histopathology, a diagnosis of RDD was performed and confirmed on IHC. This case highlights the need to create awareness among young pathologists and clinicians about PCRDD to prevent overzealous treatment.
Related Links	https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC9272694&blobtype=pdf
ISSN	2213879X
Journal	Journal of Microscopy and Ultrastructure [J Microsc Ultrastruct]
Volume Number	10
PubMed Central reference number	PMC9272694
Issue Number	2
PubMed reference number	35832315
e-ISSN	22138803
DOI	10.4103/jmau.jmau_76_20
Language	English
Publisher	Wolters Kluwer - Medknow
Publisher Date	2021-09-08
Publisher Place	India
Access Restriction	Open
Rights License	This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. Copyright: © 2021 Journal of Microscopy and Ultrastructure
Subject Keyword	Cutaneous rosai dorfman disease emperipolesis histiocytic disorder rosai dorfman disease
Content Type	Text
Resource Type	Article
Subject	Instrumentation Electronic, Optical and Magnetic Materials Pathology and Forensic Medicine Nephrology