Laryngeal ulceration in Behçet’s disease: the role of centres of excellence in the UK

Abstract

IntroductionBehçet’s disease (BD) is a rare complex multisystem inflammatory condition characterized by the triad of symptoms: aphthous ulcers of the mouth and genitalia and uveitis.Case reportWe present the case of a 31-year-old Caucasian male who had a prolonged journey from first presentation until diagnosis of BD. For 11 years, he presented symptoms affecting the oral cavity and oropharynx, with worsening odynophagia and dysphagia and ultimately, development of stridor. Flexible laryngoscopy showed significant laryngopharyngeal ulceration and scarring. Treatment was with colchicine, corticosteroids and azathioprine and supervised by one of three newly established BD Centres of Excellence in the UK.DiscussionAlthough uncommon, ENT manifestations in patients with BD should be taken into consideration to allow for early recognition and treatment of what can become a life-threatening condition. In such situations, early referral to a BD Centre of Excellence is essential to provide confirmation of diagnosis and supervision of treatment.

Legislation, Market Size, and Access to Gender-affirming Genital Surgery in the United States

“…Centers of excellence models have been proposed in an increasing number of fields, including surgical oncology, 49 bariatric surgery, 50 aortic cardiac surgery, 51 and other specialties. 52 , 53 …”

Epiglottitis in Patients With Preexisting Autoimmune Diseases: A Nationwide Case–Control Study in Taiwan

“…Inconsistent conclusions have been drawn regarding whether patients with autoimmune disease are predisposed to epiglottitis, 25 and studies investigating the autoimmune disease-epiglottitis relationship are limited. [12][13][14]16,17,26 Based on our literature review, the present research is the first populationbased study on the epiglottitis-autoimmune disease association. In working to this end, obtaining a sufficiently large sample with long observation period from only one medical institution would be difficult given the rarity of the diseases investigated.…”

“…5,7,8 Moreover, mucosa ulcer and edema of epiglottic structures, cricoarytenoid arthritis, and necrotizing vasculitis have been reported in noninfectious epiglottitis caused by autoimmune disease with laryngeal involvement. [9][10][11] Studies have highlighted the high prevalence of supraglottic involvement in patients with Sjo ¨gren syndrome (SS), Behcet disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE), 10,[12][13][14][15][16] and case reports have indicated that epiglottitis might present as the initial or flare manifestation of SLE. 11,17 Although the aforementioned studies have discovered crucial connections between the 2 rare disease entities, the lack of a control group and small samples have limited the research.…”

Laryngeal ulcer with Behcet’s disease

“…In a prior study of northern European patient cohort, 5 out of 15 patients (33%) with Behcet’s disease showed laryngeal changes 3. However, laryngeal involvement in Behcet’s disease has been reported only rarely, with complications that included acute airway obstruction, requiring surgical intervention, due to oedema 4–6. Evaluation of the airway should be considered for cases of Behcet’s disease with dysphagia.…”