Primary cutaneous Rosai-Dorfman disease; a case-based review of a diagnostically and therapeutically challenging rare variant.

Abstract:

:Primary cutaneous Rosai-Dorfman disease is a rare form of Rosai-Dorfman disease limited to the skin. The diagnosis of primary cutaneous disease is based on a combination of clinical presentation, histopathology, and the detection of S100+, CD68+, and CD1a- histiocytic immunophenotyping. However, the diagnosis of primary cutaneous disease is often difficult and significantly delayed due to the non-specific nature of its histologic and clinical features. In this review, we describe four cases in order to familiarize pathologists and dermatopathologists with the clinicopathologic correlation of primary cutaneous Rosai-Dorfman disease and to help facilitate early diagnosis. In addition, we discuss the proposed pathophysiology and molecular etiology of this tumor, and its relationship with IgG4 sclerosing disease.

journal_name

Ann Diagn Pathol

authors

Fayne R,Rengifo SS,Gonzalez I,Solorzano JL,Gonzalez D,Vega F,Cho-Vega JH

doi

10.1016/j.anndiagpath.2019.151446

subject

Has Abstract

pub_date

2020-04-01 00:00:00

pages

151446

eissn

1092-9134

issn

1532-8198

pii

S1092-9134(19)30375-2

journal_volume

45

pub_type

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