Siegel M B, Potsic W P
Department of Otolaryngology, Hospital of the University of Pennsylvania, Philadelphia 19104.
Int J Pediatr Otorhinolaryngol. 1990 Aug;19(3):265-71. doi: 10.1016/0165-5876(90)90006-d.
Ectodermal dysplasia is a rare group of diseases presenting special problems in management for the otolaryngologist, but the full spectrum of otorhinolaryngologic manifestations has been previously unrecognized in the otolaryngologic literature. The anhidrotic form, characterized by deficient sweating, sparse hair growth and deficient teeth, with associated decreased mucous production in the aerodigestive tract leads to chronic upper respiratory tract infections, otitis, dysphagia, hoarseness, bronchitis and sometimes hemoptysis.
外胚层发育不全是一组罕见疾病，给耳鼻喉科医生的治疗带来特殊问题，但耳鼻喉科文献中此前尚未认识到其耳鼻喉科表现的全貌。无汗型以外分泌汗腺功能缺陷、毛发稀疏和牙齿发育不全为特征，伴有呼吸道和消化道黏液分泌减少，可导致慢性上呼吸道感染、中耳炎、吞咽困难、声音嘶哑、支气管炎，有时还会咯血。
