Rosai-Dorfman disease with isolated laryngeal involvement

October 4, 2012 by Elisa A. Illing, MD; Stacey L. Halum, MD
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Abstract

Rosai-Dorfman disease is a rare histiocyte disorder that is typically characterized by massive cervical lymphadenopathy. Isolated extranodal involvement is uncommon, and isolated laryngeal involvement is extremely rare. We report an unusual case of Rosai-Dorfman disease with isolated laryngeal involvement that led to recurrent dysphonia and airway obstruction. We discuss the challenges we faced in reaching a correct pathologic diagnosis and in deciding on an appropriate treatment regimen. Based on our experience, we believe that Rosai-Dorfman disease should be considered as a differential diagnosis in patients who present with a recurrent inflammatory (histiocytic) mass lesion of the larynx.

Introduction Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare proliferative disorder of histiocytes. Its etiology is unclear. Clinical characteristics often include a painless cervical lymphadenopathy, fever, leukocytosis, an elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia.1

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CitationEar Nose Throat J. 2012 October;91(10):439-440