TY - JOUR
T1 - Primary cutaneous Rosai-Dorfman disease; a case-based review of a diagnostically and therapeutically challenging rare variant
AU - Fayne, Rachel
AU - Rengifo, Sandra Sanchez
AU - Gonzalez, Ivan
AU - Solorzano, Jose Luis
AU - Gonzalez, Daniel
AU - Vega, Francisco
AU - Cho-Vega, Jeong Hee
N1 - Publisher Copyright:
© 2019 Elsevier Inc.
PY - 2020/4
Y1 - 2020/4
N2 - 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.
AB - 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.
KW - Cutaneous Rosai-Dorfman disease
KW - Emperipolesis
KW - Sinus histiocytosis with massive lymphadenopathy
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U2 - 10.1016/j.anndiagpath.2019.151446
DO - 10.1016/j.anndiagpath.2019.151446
M3 - Review article
C2 - 31978810
AN - SCOPUS:85078124165
SN - 1092-9134
VL - 45
JO - Annals of Diagnostic Pathology
JF - Annals of Diagnostic Pathology
M1 - 151446
ER -