Rosai-Dorfman disease (RDD) is an uncommon histiocytic disorder typically characterized by massive lymphadenopathy and distinctive histopathological findings. Although extranodal involvement is frequently reported, critical airway compromise due to laryngotracheal infiltration remains exceptional. We describe a 41-year-old woman presenting with systemic disease involving cervical lymph nodes, nasopharynx, nasal cavities, and subglottic airway. Progressive respiratory obstruction led to tracheostomy. Despite initial treatment with systemic corticosteroids and vinblastine, inflammatory activity persisted. Introduction of weekly methotrexate resulted in radiological stabilization and gradual clinical improvement, allowing eventual decannulation. This case underscores the dual inflammatory and fibrotic mechanisms underlying airway compromise in RDD and highlights the potential role of methotrexate as a long-term immunomodulatory strategy in refractory multisystem involvement.
Afellah et al. (Wed,) studied this question.