A Rare Case of Hypohidrotic Ectodermal Dysplasia in a Seven-Year-Old Child

被引:1
作者
Shah, Syed Asfand Yar [1 ]
Ul Hassan, Wajih [1 ]
Sajjad, Waseem [1 ]
Bin Shabbir, Usama [1 ]
Raza, Ali [1 ]
机构
[1] Nishtar Med Univ, Internal Med, Multan, Pakistan
关键词
heat intolerance; hypodontia; anhidrosis; midface hypoplasia; ectodermal dysplasia; MANAGEMENT;
D O I
10.7759/cureus.24300
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Ectodermal dysplasias (EDs) encompass a large group of inherited disorders that affects two or more ectodermally derived structures. Hair, sweat glands, teeth, and nails are the most common ectodermal derivates affected. Other ectodermal structures that may be affected are ears, eyes, lips, and mucous membranes of the mouth or nose. During embryonic development, the ectoderm forms the outermost layer of the primary germ layers that give rise to the several structures that are commonly affected in ED. Therefore, ED manifests differently among patients, depending on the abnormality's combination and severity. Out of 150 distinctive syndromes, the most common syndromes within this group are hypohidrotic (defective sweat glands) and hidrotic (normal sweat glands). In addition, different types of inheritance patterns are found in ED; X-linked inheritance is by far the most common mode of inheritance. We present here the clinical case of hypohidrotic (anhidrotic) ED in a seven-year-old boy.
引用
收藏
页数:5
相关论文
共 18 条
[1]  
Bari AU, 2007, J PAK ASS DERMATOL, V17, P52
[2]   SWEATING IN ECTODERMAL DYSPLASIA SYNDROMES - A REVIEW [J].
BERG, D ;
WEINGOLD, DH ;
ABSON, KG ;
OLSEN, EA .
ARCHIVES OF DERMATOLOGY, 1990, 126 (08) :1075-1079
[3]  
Bonilla E D, 1997, Quintessence Int, V28, P657
[4]  
BORG P, 1977, J DENT CHILD, V44, P34
[5]   Clinical findings in mosaic carriers of hypohidrotic ectodermal dysplasia [J].
Cambiaghi, S ;
Restano, L ;
Pääkkönen, K ;
Caputo, R ;
Kere, J .
ARCHIVES OF DERMATOLOGY, 2000, 136 (02) :217-224
[6]  
Deshmukh Seema, 2012, Int J Clin Pediatr Dent, V5, P197, DOI 10.5005/jp-journals-10005-1165
[7]  
Ectodermal Dysplasia Society, 2022, US
[8]   Dental manifestations of dermatologic conditions [J].
Freiman, Anatoli ;
Borsuk, Daniel ;
Barankin, Benjamin ;
Sperber, Geoffrey H. ;
Krafchik, Bernice .
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 2009, 60 (02) :289-298
[9]  
Freire-Maia N, 1988, Birth Defects Orig Artic Ser, V24, P3
[10]   Prenatal Therapy in Developmental Disorders: Drug Targeting via Intra-Amniotic Injection to Treat X-Linked Hypohidrotic Ectodermal Dysplasia [J].
Hermes, Katharina ;
Schneider, Pascal ;
Krieg, Peter ;
Anh Thu Dang ;
Huttner, Kenneth ;
Schneider, Holm .
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 2014, 134 (12) :2985-2987