Soonchunhyang Medical Science  2014;20(1):31-34

Rosai-Dorfman Disease Combined with Aortic Vasculitis, Arrhythmia, and Valvular Heart Disease.

Shin Il KIM 1 ; Ji Young HAN ; Young Hoon PARK ; Joo Han LIM ; Moon Hee LEE ; Chul Soo KIM ; Hyeon Gyu YI

Affiliations

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Keywords

Rosai-Dorfman disease; Vasculitis; Cardiac arrhythmia; Heart valve diseases; Prednisolone; Methotrexate

Country

Republic of Korea

Language

English

Abstract

Rosai-Dorfman disease (RDD) is a rare and benign self-limited disorder with pathologic feature of the lymph node sinuses expanded by a proliferation of distinctive histiocytes. The most often involving site is bilateral cervical lymphadenopathy, followed by the skin and soft tissue. Treatment options, including steroid, chemotherapy, radiotherapy and debulking surgery depend on the symptoms or the extent and localization of the lesions. We encountered a very rare case of RDD at the skin lesions, particularly combined with aortic vasculitis, arrhythmia, and valvular heart disease.