PDF(Pubmed)
Abstract：
ECTODERMAL DYSPLASIA (ED) IS A RARE DISORDER WITH DEFECTS IN TWO OR MORE OF THE FOLLOWING STRUCTURES: the teeth and the skin and its appendages including hair, nails, eccrine, and sebaceous glands. Dental manifestations include hypodontia, complete anodontia or malformed teeth. The most common form of the ED syndrome is hypohidrotic ED and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. The condition is thought to occur in approximately 1 in every 100,000 live births. Dental treatment for these patients varies on an individual basis. Children with ED are often treated dentally with conventional adult appearing prosthesis which are focused only on the oral manifestations of the syndrome. We are here reporting two classical cases of hypohidrotic ED with a review of the literature.
摘要：
外皮发育不良（ED）是一种罕见的疾病，在以下两个或多个结构中存在缺陷：牙齿和皮肤及其附属物，包括头发，指甲,eccrine,和皮脂腺.牙齿表现包括牙体发育不全，完全无牙齿或牙齿畸形。ED综合征最常见的形式是多汗症性ED，通常以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。据认为，每100,000例活产中大约有1例发生这种情况。这些患者的牙科治疗因人而异。患有ED的儿童通常使用传统的成人外观假体进行治疗，这些假体仅关注该综合征的口腔表现。我们在这里报道了两例经典的低汗症ED病例，并对文献进行了回顾。
