Lingual Raynaud's Phenomenon: A Case Series and Literature Review.

Background: Raynaud's phenomenon of the tongue is a rare manifestation that may be associated with systemic diseases. The clinical manifestations, etiologies and management of this condition are poorly described. Methods: We report 10 cases of lingual Raynaud's phenomenon (LRP) and 26 cases from a structured literature review. Results: In 38.8% of cases, the LRP occurred in the context of a previously diagnosed systemic sclerosis; 16.6% followed radiotherapy for head and neck cancer; and 27.8% of patients presented with an idiopathic-like form. The manifestations classically included a syncopal phase (91.7%) associated with hypoesthesia (88.9%) and possible dysarthria (52.8%). Atypical presentations with a primary cyanotic phase were also observed, particularly in the context of vasculitis, notably cryoglobulinemic vasculitis (four patients). Active smoking was a significant triggering factor in idiopathic forms (60%). Across all patients-both primary and secondary forms-the most common triggering factor was cold exposure (75%). Vasodilator use showed good efficacy and should be considered for all highly symptomatic patients. Conclusions: In summary, LRP is more frequently associated with systemic sclerosis, manifesting as blanching of the tongue associated with hypoesthesia and dysarthria in more than half of cases. Vasodilators may reduce symptoms. Larger studies are needed to confirm these findings.