© 2017 Indian Dermatology Online Journal | Published by Wolters Kluwer Medknow Ectodermal dysplasias are a heterogeneous group of disorders resulting from abnormalities of structures derived from embryonic ectoderm (skin, teeth, and appendage). Rapp Hodgkin Syndrome (RHS) is a type of anhidrotic ectodermal dysplasia, which was described by Rapp and Hodgkin in 1968.[1] This syndrome is character...

The etiology of tooth agenesis may be related to several factors, among them, the genetic alterations that play a fundamental role in the development of this dental anomaly, so that knowledge about it helps the clinician to have a greater understanding of their patients. Thus, the aim of this study was to report the case of a nonsyndromic child, with tooth agenesis of one premolar, three first ...

CASE REPORT This report describes the oral rehabilitation of a young boy with severe oligodontia of primary dentition and complete anodontia of permanent dentition, treated by complete maxillary and partial mandibular removable dentures. Since the determination of a proper occlusal vertical dimension in paediatric patients is often difficult, treatment was achieved by means of a multidisciplina...

Hypohidrotic (anhidrotic) ectodermal dysplasia (HED) is a congenital syndrome characterized by sparse hair, oligodontia, and reduced sweating. It is estimated to affect at least one in 17000 people worldwide. We report a rare case of HED in a 14-year-old male child patient which extraorally manifested as alopecia, scanty eyebrow and eye lashes, frontal bossing, depressed nasal bridge, and full ...

Correspondence to: Guna Shekhar c/o M.Prakash, 160, 5th Cross, 28th main, sector-1, HSR Layout, Bangalore-102, INDIA E-mail: [email protected] Received for publication: December 04, 2009 Accepted: March 04, 2010 Abstract Hypohidrotic ectodermal dysplasia (HED) is a hereditary syndrome, characterized by a classic triad of hypotrichosis, hypodontia and hypohidrosis. The case of an 8-year-old g...

Ectodermal dysplasia is a heterogeneous condition characterized by affected ectodermal structures, among which the teeth and skin with its derivatives (hair sweat glands) are the most frequent. The aim of this work is to present the analysis of dental traits in five families (affected boys and their mothers) with hypohidrotic ectodermal dysplasia (HED), and to evaluate the importance of orofaci...

Dental implants have become an accepted treatment modality for aging patients with either completely or partially edentulous arches. However, growing patients with congenitally missing primary and/or permanent teeth often need dental implant treatment, even before puberty, for optimum functional and/or psychosocial development. From a developmental perspective, dental implants cannot accompany ...

Individuals with cleft lip and palate present significantly more dental anomalies, even outside the cleft area, than do individuals without clefts. Our aim was to evaluate the prevalence of tooth agenesis and patterns of hypodontia in a large sample of patients with complete bilateral cleft lip and palate (BCLP). Serial panoramic radiographs (the first radiograph was taken at 10.5-13.5 yr of ag...

Although hypodontia, or oligodontia, is one of the most common human dental anomalies observed, there have been few studies on the association of other anomalies occurring with it. The present investigation of 1032 patient records found that 65.7% of patients with hypodontia showed ankylosis of primary molars compared to only 1.5% of control children (P less than 0.001). In addition, taurodonti...

Interstitial deletions of chromosome 12p are rare, and the phenotype spectrum is therefore still unknown. The thirteen patients reported so far suffer from developmental delay, optic nerve hypoplasia, micropenis, hypoplastic hair and skin, oligodontia, brachydactyly, and arterial hypertension. We report a de novo 12p12.2-p11.22 deletion of 9.2 Mb detected by array CGH analysis in a boy with glo...