Rosai-Dorfman disease of the lung with features of obliterative arteritis

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare benign non-Langerhans cell histiocytosis. Twenty-five to forty percent of the cases are extranodal and have been reported in virtually all anatomic locations. In this article, we report a highly unusual case of RDD which presented as multiple pulmonary nodules and associated hilar lymphadenopathy. On resection, extensive obliterative arteritis was noticed. This pathologic presentation of RDD has not been reported before in the English literature. Accurate recognition of this entity is crucial to prevent unnecessary aggressive treatment.