the ectodermal dysplasia represents a group of inherited conditions in which two or more ectodermally derived anatomic structures fail to develop .
ectodermal dysplasias represent a large and complex group of diseases comprising of more than 170 clinical conditions .
depending upon the presence or absence of sweat glands , it is divided into the hidrotic ( clouston syndrome ) and anhidrotic types .
( cst - syndrome i.e. christ- siemen - touraine syndrome and anhidrotic / hypohidrotic , ectodermal dysplasia being synonymous . ) in most cases , this disorder seems to show an x - linked pattern , with gene mapping to xq12-q13.1 ; therefore , a male predominance is usually seen .
individuals affected by it show the triad comprising anhydrosis / hypohydrosis , hypotrichosis , and dental hypoplasia .
hypodontia has been considered to be a multifactorial condition with genetic and environmental influences , and published opinions differ on the importance of each factor .
larmour et al . , state that recent developments in molecular genetics are revealing the roles of the homeobox genes in the control of the complex epithelial / mesenchymal interactions that occur during dental development .
those of particular interest for dental development are the muscle - specific homeobox genes , msx1 and msx2 .
here is the case report of a 8-year - old male child with ectodermal dysplasia .
the chief complaint of the patient was difficulty in mastication due to absence of maxillary and mandibular teeth .
the patient experienced episodes of high fever , was intolerant to heat , and did not sweat .
his i.q . , level was low , and it was evident when asked about schooling .
other characteristics of ectodermal dysplasia , such as frontal bossing , saddle nose , reduced vertical dimension of face due to total anodontia were also noticed .
child showing dry skin , sparse eyebrows , eyelashes , and scalp hair hands showing dystrophic ( thin and brittle ) nails feet showing dry skin and dystrophic nails intraoral examination revealed absence of teeth with thin alveolar crests [ figures 4 and 5 ] .
complete anodontia of maxillary arch complete anodontia of mandibular arch occlusal and panoramic radiographs revealed no primary and permanent teeth [ figures 68 ] . in order to improve mastication and aesthetics , both upper and lower complete dentures were fabricated [ figure 9 ]
occlusal view of maxilla occlusal view of mandible orthopantomogram ( opg ) showing complete anodontia post treatment photograph
ectodermal dysplasia is one of the most important anomalies of interest to dental clinicians because of the absent or misshapen teeth .
hypodontia is known as one of the major factors of ectodermal dysplasia and is almost always present . in severe cases , no teeth form .
the absence of primary teeth ( true anodontia ) is a rare phenomenon . in this case , the patient 's history and clinical and radiographic examination revealed the absence of primary teeth .
acikgoz et al . , and vieira et al . , also reported true anodontia of primary teeth .
total anodontia denoted by complete developmental absence of teeth in both primary and secondary dentitions was reported pirgon et al . and pannu and singh .
it is claimed that primary teeth must be present for the development of their permanent successors .
there are no permanent teeth in the oral cavity of the patient , similar finding reported by vieira et al .
the patient experienced episodes of high fever , was intolerant to heat , and did not sweat .
child 's inability to perspire , more comfortable during cold weather , and absence of hair from the eyebrows with scanty eyelashes were also reported by gupta et al . and pannu and singh ( 2002 ) .
short stature , underweight in relation to age and mental retardation were reported in accordance to case reported by gupta .
management : to improve the appearance , mastication , and speech , the child was provided with maxillary and mandibular complete dentures similar to treatment provided by vierra et al .
the dental team should be aware of the clinical presentation of ectodermal dysplasia in order to provide the correct guidance for functional , social , and psychological needs of the patients .
 the hereditary condition known as ectodermal dysplasia is characterized by the absence or defect of two or more ectodermally derived structures . 
 the most commonly observed forms of ectodermal dysplasia are the hidrotic and hypohidrotic types ; discrimination is based on the absence or presence of sweat glands . 
 a case of 8-year - old male child with hypohidrotic ectodermal dysplasia with complete anodontia of primary as well as secondary dentitions is presented . 
 the child had a short stature , low intelligent quotient ( i.q . 
 , ) , and was underweight . 
 the patient experienced episodes of high fever , was intolerant to heat , and did not sweat . 
 he exhibited smooth and dry skin , sparse light - colored eyebrows . 
 dental clinicians can be the first to diagnose ectodermal dysplasia due to the absence of teeth . 
INTRODUCTION
CASE REPORT
DISCUSSION