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Rosai-Dorfman Disease : Introduction

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Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare, benign, self-limiting histiocytic disorder whose pathogenesis is poorly understood. It is characterized by accumulation of abnormal histiocytes undergoing emperipolesis and causing sinusoidal dilation (when involving lymph nodes).

It usually presents as massive bilateral enlargement of cervical lymph nodes accompanied by fever and weight loss. Other lymph node groups or extranodal sites (most commonly skin and CNS) may be involved.

Grossly, the involved lymph nodes are massively enlarged and show white-tan, homogenous, solid cut surface.

Image courtesy of: Dr. Luis Humberto Cruz Contreras, Mexico; used with permission.

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