Bhargava Akshay, Sharma Arun, Popli Sachet, Bhargava Renu
J Indian Prosthodont Soc. 2010 Jun;10(2):137-40. doi: 10.1007/s13191-010-0026-9. Epub 2010 Dec 8.
Ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. Children with ectodermal dysplasia may have various manifestation of the disease that differ in severity and may or may not involve teeth, skin, hair, nails, sweat glands and sebaceous glands. The most common form of the ectodermal dysplasia syndrome is hypohidrotic ectodermal dysplasia and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. This case report discusses the management of a 5-year-old girl with ectodermal dysplasia. Clinical management consisted of fabricating upper and lower dentures to help in psychosocial development and to restore the vertical dimension, esthetics and functioning of the stomatognathic system.
外胚层发育不全是一种罕见的先天性疾病,会影响多个外胚层结构。患有外胚层发育不全的儿童可能有该疾病的各种表现,严重程度各异,可能累及或不累及牙齿、皮肤、毛发、指甲、汗腺和皮脂腺。外胚层发育不全综合征最常见的形式是少汗型外胚层发育不全,通常作为X连锁隐性性状遗传。女性携带者可能有不同程度的临床表现。本病例报告讨论了一名5岁外胚层发育不全女童的治疗。临床治疗包括制作上下假牙,以帮助其心理社会发展,并恢复口腔颌面部系统的垂直距离、美观和功能。