Destombes-Rosai-Dorfman disease: A case series of nodular and extra nodular involvement

Abstract

Introduction and importanceRosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder characterized by painless lymph node enlargement and extranodal involvement. This disease often presents diagnostic challenges due to its variable clinical manifestations and the need for histologic confirmation. Case series presentationThis case series examines five patients diagnosed with Rosai-Dorfman disease who presented with a variety of clinical manifestations. Patients ranged in age from 21 to 72 years and presented with a variety of symptoms including cervical lymphadenopathy, mediastinal adenopathies, thyroid nodules, and nasal obstruction. Diagnoses were confirmed by histologic examination and immunostaining, primarily with anti-PS100 antibody. Treatments included surgery, corticosteroids, and laser resection, with most cases showing favorable outcomes and no recurrence at follow-ups ranging from one to five years. Clinical discussionThe diverse presentations of RDD in this case series underscore the importance of considering this entity in the differential diagnosis of lymphadenopathy and other mass lesions. Histologic examination and immunostaining are critical for accurate diagnosis. ConclusionThis case series aims to highlight the diverse presentations, diagnostic approaches, and treatment outcomes of patients with Rosai-Dorfman disease and to contribute to a better understanding of this rare condition.