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

Rachel Fayne, Sandra Sanchez Rengifo, Ivan Gonzalez, Jose Luis Solorzano, Daniel Gonzalez, Francisco Vega, Jeong Hee Cho-Vega

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

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.

Original languageEnglish (US)
Article number151446
JournalAnnals of Diagnostic Pathology
Volume45
DOIs
StatePublished - Apr 2020

Keywords

  • Cutaneous Rosai-Dorfman disease
  • Emperipolesis
  • Sinus histiocytosis with massive lymphadenopathy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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