長期経過観察している瘢痕性類天疱瘡の女児

概要

論文の詳細を見る
Cicatricial pemphigoid is a rare autoimmune disease with chronic blister and decomposition formation in the membrane leaving scarring. Blistering and decomposition may also be expected in the pharynx, larynx or esophagus. This study began when the subject started showing symptoms from 3 years of age and continued for 10 years as the cicatricial pemphigoid progressed. Breathing difficulty appeared nightly when she was 3 years and 10 months old. It was urgent, requiring endotracheal intubation and a tracheotomy. Rubor of a left main bronchus and strangulation effects were observed from 4 and a half years of age. A keloplasty of the epiglottis was performed and the glottis was closed by cicatricial adhesion. In Japan, the number of cicatricial pemphigoid cases requiring tracheotomies, including this case, was 15. The number of cases whose tracheal gill holes were closed relatively quickly was 3. Medical treatment of the larynx cannot be carried out while the condition is not under control. The girl in this study is now a junior high school student and has the ability for satisfactory everyday conversation as well as functional deglutition. Since steroids are prescribed for ongoing treatment, we think its important to see and carefully monitor progress. Depending on future progress, it may be that larynx reconstruction among other physical treatments could be indicated.