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Two cases of linear IgA bullous dermatosis initially presenting as ulcerative lesions in the larynx and pharynx are reported. It was difficult to diagnose and treat the lesions, but they were finally diagnosed from the histopathological findings of accompanying skin lesion specimens. One of the patients required a tracheostomy due to increased airway stenosis by a laryngeal lesion. Despite general corticosteroid administration this could not be completely resolved, although partial opening of the glottis was observed, and the patient died of accidental tracheostomy tube complications during home care. Although there are no reports of this disease in the otolaryngological field, these rare diseases involving the skin and entire body should be considered in the differential diagnosis of laryngeal and pharyngeal ulcerative lesions, including airway stenosis. Furthermore, simple and safe procedures for relieving airway stenosis should be selected for rare and difficult-to-diagnose airway disease, prior to the final diagnosis.

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