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Supraglottic stenosis in a case of Behcet’s disease

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Behcet’s disease (BD) is an inflammatory systemic disease characterized by recurrent oral aphthous ulcers (OAUs), genital aphthous ulcers, cutaneous lesions, and uveitis. OAUs occur mainly in the oral cavity (1).… Click to show full abstract

Behcet’s disease (BD) is an inflammatory systemic disease characterized by recurrent oral aphthous ulcers (OAUs), genital aphthous ulcers, cutaneous lesions, and uveitis. OAUs occur mainly in the oral cavity (1). Aphthous ulceration is rarely reported in other parts of the aerodigestive tract, such as the hypopharynx, larynx, and vocal cords (2). Aphthous ulcerations in the aerodigestive tract almost always heal without scarring but, in very rare cases, large ulcers produce scars. Supraglottic stenosis is a rare subset of laryngotracheal stenosis. In a large retrospective study, 3% of patients with laryngotracheal stenosis had supraglottic stenosis (3). Symptoms of supraglottic stenosis in adults include dysphagia, inspiratory stridor, resting and exertional dyspnoea, and changes in the voice (4). Stevens et al, in a case series study on eight patients, reported that the most common cause of laryngotracheal stenosis (62.5%) is radiation injury (4). The remainder of their patients had autoimmune disease (37.5%). In 1951, Kenet reported the first case of laryngeal involvement in BD, which was resistant to treatment with steroids and needed repeated tracheotomies (5). Since then, only a few cases of laryngeal stenosis in BD have been described. Table 1 shows the published cases of laryngeal involvement in BD. In this article, we report a case of BD with supraglottic laryngeal stenosis. A 43-year-old womanwith knownBDwas admitted with dysphagia and stridor in June 2016. Her disease had presented 10 years previously with recurrent oral and genital aphthous ulcerations and pseudofolliculitis. She had been referred to the Connective Tissue Diseases Research Center (CTDRC) clinic in September 2014 because of the recurrent OAUs. Pathergy test was positive, so treatment with colchicine was started after diagnosing BD. Since her symptoms did not improve, treatment with prednisolone 7.5 mg/day andmethotrexate 10mg/weekwas started. However, she did not follow the treatment and discontinued her medications. In June 2016, she was referred to the CTDRC again because of the changes in her voice, lack of appetite, and mild dysphagia. On examination, six minor aphthous ulcers were observed in the tongue, soft palate, and pharynx. The patient had inspiratory stridor and her voice was nasalized. Other systemswere normal on examination. Laboratory tests were as follows: white blood cell count 6.71 × 10/mm, haemoglobin 11.5 g/dL, erythrocyte sedimentation rate 32 mm/h, C-reactive protein 11 mg/dL (0-6 mg/dL), aspartate transaminase 22 IU/L, alanine transaminase 28 IU/L, blood urea nitrogen 20 mg/dL, creatinine 0.8 mg/dL, human leucocyte antigen (HLA)-B5 positive, HLA-B51 positive, anti-nuclear antibody negative, and urine analysis normal.

Keywords: behcet disease; treatment; supraglottic stenosis; disease; case; stenosis

Journal Title: Scandinavian Journal of Rheumatology
Year Published: 2018

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