Abstract
Cicatrical pemphigoid is a rare autoimmune blistering disease with a chronic relapsing disease course. involving predominantly mucous membranes that often results in scar formation in elderly patients. We report an 81-year-old Japanese male patient with anti-epiligrin cicatrical pemphigoid, who showed a severe laryngeal stenosis which necessitated a tracheostomy. Later, cutaneous bullous lesions appeared on the whole body. Direct immunofluorescence showed deposition of IgG and C3 along the basement membrane zone. The diagnosis was made by the results of immunofluorescence and immunoprecipitation studies. He was treated with combination of tetracycline 1,500mg/day and nicotinamide 1,500mg/day, and skin lesions disappeared quickly. Because dyspnea disappeared in two months, the tracheal stoma was closed under local anesthesia. He showed no respiratory difficulty for 7 month follow-up period.
