Kaul S, Reddy R
MDS (Prosthodontics), ITS-CDSR, Ghaziabad, Uttar Pradesh, India.
J Indian Soc Pedod Prev Dent. 2008 Dec;26(4):177-81. doi: 10.4103/0970-4388.44041.
Ectodermal dysplasia is a hereditary syndrome characterized by dysplasia of tissues of ectodermal origin (hair, skin, nails, and teeth) and occasionally, dysplasia of mesodermally derived tissues. The triad of nail dystrophy (onychodysplasia), alopecia, or hypotrichosis (scanty, fine, light hair on the scalp and eyebrows) and palmoplantar hypohidrosis is usually accompanied by lack of sweat glands and partial or complete absence of primary and permanent dentition. Hypohidrotic ectodermal dysplasia usually has an X-linked inheritance and affects only males severely, while female heterozygotes show only minor defects. The clinical management of children with ectodermal dysplasia provides a unique opportunity for cooperative effort between the pedodontist and the prosthodontist. The following case report discusses the management of a young boy with hypohidrotic ectodermal dysplasia. Removable prostheses were employed in the treatment. The aim was to rehabilitate the adolescent prosthodontically and boost him psychologically.
外胚层发育不良是一种遗传性综合征，其特征为外胚层起源的组织（毛发、皮肤、指甲和牙齿）发育异常，偶尔也有中胚层来源组织的发育异常。甲营养不良（甲发育异常）、脱发或毛发稀少（头皮和眉毛上稀疏、纤细、浅色的毛发）以及掌跖少汗三联征通常伴有汗腺缺失以及乳牙和恒牙部分或完全缺失。少汗型外胚层发育不良通常具有X连锁遗传，仅严重影响男性，而女性杂合子仅表现出轻微缺陷。外胚层发育不良患儿的临床管理为儿童牙医和口腔修复医生的合作提供了独特的机会。以下病例报告讨论了一名少汗型外胚层发育不良小男孩的治疗情况。治疗中采用了可摘义齿。目的是通过口腔修复使这名青少年恢复功能，并在心理上给予他支持。
