Luke M C, Darling T N, Hsu R, Summers R M, Smith J A, Solomon B I, Thomas G R, Yancey K B
Dermatology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
Arch Dermatol. 1999 Aug;135(8):954-9. doi: 10.1001/archderm.135.8.954.
Epidermolysis bullosa acquisita is an acquired inflammatory and/or dermolytic subepidermal blistering disease characterized by IgG autoantibodies to type VII collagen. Four patients with documented epidermolysis bullosa acquisita were evaluated by a multidisciplinary team of care providers (4 dermatologists, an ophthalmologist, a radiologist, a voice and speech specialist, and an otolaryngologist) for 1 to 5 years to characterize mucosal involvement and its complications and response to treatment. Patients were evaluated clinically and by slitlamp examinations, endoscopies, computed tomographic scans, and videofluorographic swallowing studies. Spiral computed tomographic scans for virtual endoscopy were used for the nontraumatic evaluation of airways in 2 patients with respiratory tract compromise.
Involvement of 5 or more mucosal sites--mouth, nose, conjunctiva, pharynx, and larynx--was documented in all patients. Complications included ankyloglossia, periodontal disease, scarring and crusting of nasal mucosa, symblepharon formation, obstruction of nasolacrimal ducts, deformation of the epiglottis, impaired phonation, dysphagia, esophageal strictures, and supraglottic stenosis requiring emergency tracheostomy.
Epidermolysis bullosa acquisita may extensively (or predominantly) affect mucosal epithelia in a manner resembling cicatricial pemphigoid. Mucosal disease in these patients is often subclinical, can lead to serious complications, and is best managed using a multidisciplinary approach.
获得性大疱性表皮松解症是一种获得性炎症性和/或皮肤溶解性表皮下大疱病，其特征为针对VII型胶原的IgG自身抗体。一个多学科护理团队（4名皮肤科医生、1名眼科医生、1名放射科医生、1名语音和语言专家以及1名耳鼻喉科医生）对4例有记录的获得性大疱性表皮松解症患者进行了1至5年的评估，以确定黏膜受累情况及其并发症和对治疗的反应。对患者进行了临床评估，并通过裂隙灯检查、内镜检查、计算机断层扫描和视频荧光吞咽研究进行评估。对2例呼吸道受损患者使用螺旋计算机断层扫描进行虚拟内镜检查，以对气道进行非创伤性评估。
所有患者均有5个或更多黏膜部位（口腔、鼻腔、结膜、咽和喉）受累的记录。并发症包括舌系带过短、牙周病、鼻黏膜瘢痕形成和结痂、睑球粘连形成、鼻泪管阻塞、会厌变形、发声障碍、吞咽困难、食管狭窄以及需要紧急气管切开术的声门上狭窄。
获得性大疱性表皮松解症可能以类似于瘢痕性类天疱疮的方式广泛（或主要）影响黏膜上皮。这些患者的黏膜疾病通常为亚临床状态，可导致严重并发症，最好采用多学科方法进行管理。
