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Stomach and Intestine(Tokyo) Volume 50, Issue 2 （February 2015）

胃と腸 50巻2号（2015年2月発行）

Japanese English

今月の主題食道のびらん・潰瘍性病変

主題

自己免疫性水疱症と上部消化管病変の内視鏡診断 Endoscopic Findings Observed in Pharynx and Esophagus of Autoimmune Bullous Diseases 大森泰 ¹ , 石井賢二郎 ² , 岡村明彦 ³ , 筒井麻衣 ⁴ , 中村理恵子 ³ , 川久保博文 ³ , 山上淳 ⁵ , 天谷雅行 ⁵ , 北川雄光 ³ Tai Omori ¹ , Kenjiro Ishii ² , Akihiko Okamura ³ , Mai Tsutsui ⁴ , Rieko Nakamura ³ , Hirofumi Kawakubo ³ , Jun Yamakami ⁵ , Masayuki Amagai ⁵ , Yuko Kitagawa ³ ¹川崎市立井田病院内視鏡治療部・外科 ²静岡赤十字病院外科 ³慶應義塾大学医学部一般消化器外科学教室 ⁴川崎市立川崎病院外科 ⁵慶應義塾大学医学部皮膚科学教室 ¹Department of Endoscopy, Kawasaki Municipal Ida Hospital, Kawasaki, Japan ²Department of Surgery, Japanese Red Cross Shizuoka Hospital, Shizuoka, Japan ³Department of Surgery, School of Medicine, Keio University, Tokyo ⁴Department of Surgery, Kawasaki Municipal Kawasaki Hospital, Kawasaki, Japan ⁵Department of Dermatology, School of Medicine, Keio University, Tokyo キーワード：自己免疫性水疱症 , 天疱瘡 , 類天疱瘡 , 内視鏡 , 粘膜病変 Keyword: 自己免疫性水疱症 , 天疱瘡 , 類天疱瘡 , 内視鏡 , 粘膜病変 pp.159-173

発行日 2015年2月25日 Published Date 2015/2/25

DOI https://doi.org/10.11477/mf.1403200147

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要旨●自己免疫性水疱症は，自己抗体により細胞間接着が障害され，皮膚や重層扁平上皮粘膜に水疱が形成される疾患の総称である．天疱瘡群と類天疱瘡群に分けられ，推計罹患者数は天疱瘡群約5,500人，類天疱瘡群約20,000〜30,000人とされる．水疱症には皮膚病変がなく粘膜病変のみが認められる症例も存在し，このような症例では咽頭痛・嚥下障害などにより内視鏡検査が施行されるが，まれな疾患であるために粘膜病変から確定診断に至らず治療が遅延することがある．消化器医も自己免疫性水疱症の適切な知識を持ち，的確な内視鏡診断を行うことで臨床診断に寄与できる．上部消化管内視鏡検査を行った自己免疫性水疱症93例の検討から，自己免疫性水疱症の上部消化管病変について報告する．

　Autoimmune bullous dermatosis is the general term for the disease caused by dysfunctional intercellular adhesion, which results in the formation of bullae. Some patients with bullous dermatosis have no epidermal lesions and only present with mucosal lesions. These patients undergo endoscopy because of pharyngeal pain or dysphagia, but because of the rarity of the disease, a definitive diagnosis cannot be achieved from the mucosal lesions, and treatment is sometimes delayed. Gastroenterologists are required to have relevant knowledge of autoimmune bullous dermatosis and to conduct an accurate endoscopic diagnosis. We investigated upper gastrointestinal tract lesions in 93 patients with autoimmune bullous dermatosis by performing upper gastrointestinal tract endoscopy.

　Patients with autoimmune bullous dermatosis had mucosal lesions in the oropharynx and esophagus, and the discovery rate of these lesions was 46.2%.

　Characteristic lesions included painful irregular ulcers, erosions, and edema of oropharynx; bulla formation or irregular shallow ulcers was also observed. Nikolsky's sign was also present during examination of the oropharynx and esophagus in patients with autoimmune bullous dermatosis, as evidenced in particular by characteristic findings such as the formation of bullae and blood blisters due to contact with the endoscope, epithelial desquamation, bullae and blood blister formation surrounding biopsy sites, as well as epithelial desquamation following abrasion. Gastroenterologists are thus required to have relevant knowledge of autoimmune bullous dermatosis to facilitate early diagnosis.