ectodermal dysplasia ( ed ) syndrome is a large , heterogeneous group of inherited disorders , the manifestations of which could be seen in more than one ectodermal derivative . these tissues primarily are the skin , hair , nails , eccrine glands , and teeth . several ed syndromes may manifest in association with midfacial defects , mainly cleft lip and palate . in 1961 , rosselli and gulienetti reported four patients with hypohidrosis , hypotrichosis , microdontia , dystrophic nails , cleft lip and palate , deformities of the extremities , and malformations of the genitourinary system . and freire - maia in 1970 reported a very similar clinical condition and described it as eec syndrome that is ectrodactyly  ed  cleft lip / palate syndrome . we report a case of 10-year - old male child with eec syndrome , presented as ectrodactyly , ed , cleft lip and palate , mental retardation , and hearing loss . a 10-year - old male patient reported to the department of pedodontics with the chief complaint of multiple decayed teeth since past 2 years . the medical history revealed operated cleft lip of left side , intolerance to heat , and less sweat production . family history : there was no history of consanguineous marriage between the parents . in prenatal history , no information worth mentioning was obtained . on extraoral examination , the patient had dry skin with sparse hair on the body and scalp . syndactyly of middle and ring finger was present in the right hand and ectrodactyly of index finger of right hand , third and fourth toes of right feet was also appreciated [ figures 2 and 3 ] . there was impairment of hearing but because of lack of cooperation , audiometric analysis could not be performed . the speech defect was attributed to anatomical lip and palate impairment and to his psychomotor retardation . ectrodactyly of index finger , syndactyly middle and ring finger of right - hand and dystrophic nails ectrodactyly of third , fourth toes of right feet intraoral examination showed rotated 21 , missing 12 and 22 , carious 54 , 55 , 16 , 63 , 64 , 65 , 26 , 74 , 36 , 84 , 85 , 46 [ figures 4 and 5 ] . an orthopantomograph was made which revealed congenitally missing maxillary lateral incisors , maxillary left permanent canine , both maxillary second premolars , and mandibular right second premolar . taurodontism present involving all first and second deciduous molars and first permanent molars [ figure 6 ] . the first published report of a patient with ed was in 1848 by thurman , but the term  ectodermal dysplasia  was coined by weech in 1929 . weech aa suggested the term  hereditary ectodermal dysplasia  and described three essential features , which are as follows : ( a ) tissues affected are principally of ectodermal origin , ( b ) the defects are developmental anomalies , and ( c ) the hereditary tendencies are strongly developed . the first classification system for eds was proposed by pinheiro and freire - maia in 1982 . the patients are classified into subgroups based on the presence or absence of the following : ( 1 ) trichodysplasia , ( 2 ) dental abnormalities , ( 3 ) onychodysplasia , and ( 4 ) dyshidrosis . overall , eds were classified into either group a disorders , which were manifested by defects in at least two of four classic ectodermal structures as defined above ( 1 to 4 ) , with or without other defects , and group b disorders were manifested by a defect in one classic ectodermal structure , in combination with a defect in one other ectodermal structure , i.e. , ears , lips , and dermatoglyphics . a condition characterized by only ectodermal signs is called a pure ectodermal dysplasia ; if it combines ectodermal signs and malformations , it is termed as ectodermal dysplasia malformation syndrome or an ed syndrome . our case belongs to ed syndrome category . eec syndrome ( ectrodactyly , ed , and cleft lip / palate ) is a relatively common syndrome which includes most of the manifestations of the syndactyly , ed , and cleft lip / palate syndrome . it is a complex pleiotrophic multiple congenital anomaly / dysplasia syndrome in which any one of the cardinal signs is present in variable expression . eec syndrome may exist in two forms : one with cleft lip with or without cleft palate and with cleft palate alone . both forms are inherited as autosomal dominant traits with variable expressivity , involving partial penetrance in the former and complete penetrance in the latter . the individual frequency of ectrodactyly is reported to be 1.5 per 100,000 live births and 1 per 100,000 live births for cleft palate with or without cleft lip . the occurrence of all three disorders in one , i.e. , ectrodactyly , ed , and cleft lip / palate is reported to be approximately 1.5 per 100 million . in our case , ectrodactyly was present with respect to right - hand index finger , along with the syndactyly of right - hand middle and ring finger and third , fourth toes of right foot . the syndactyly involved right - hand fingers and toes , but the function of extremities was not profoundly affected though cases reported by most of the other authors have had more severe limb defects . it has been suggested that the eec syndrome with complete and incomplete forms and other similar conditions form a family  of different although very similar syndromes , as suggested by pinheiro and freire - maia . our case showed similar problems as that of bowen and armstrong showing syndactyly , ed with abnormalities of scalp hair , and teeth and hidrotic problems , but with unilateral cleft lip and palate instead of bilateral cleft lip and palate . we suggest that our case represents either a variant of eec syndrome or a new syndrome with autosomal recessive inheritance . sparse and kinky hair with small , carious teeth , and broad nose and a prominent chin gave a typical facial appearance to our patient , similar to cases of brill et al . in addition , our case had congenitally missing both upper and lower right second premolars , missing upper permanent lateral incisors , missing upper left permanent canine , and taurodontism in all deciduous molars and first permanent molars . in our case , there was congenital absence of teeth unrelated to alveolar ridge clefting and the same was reported by bowen and armstrong 1976 . 1970 and brill et al . ) but appears to be infrequent and the same was present in our case . ectrodactyly  ed  cleft lip / palate ( eec syndrome ) is a rare form of ed , the symptoms of which can vary from mild to severe . the most common symptoms found in patients with eec syndrome are as follows : missing or irregular fingers and/or toes ( ectrodactyly ) , abnormalities of hair and glands , cleft lip and/or palate , or unusual facial features , as well as abnormalities of eyes and urinary tract . for accurate diagnosis of patients with this rare syndrome
the ectodermal dysplasias ( eds ) are a large and complex group of diseases . 
 more than 170 different clinical conditions have been recognized and defined as ectodermal dysplasias . 
 commonly involved ectodermal - derived structures are hair , teeth , nails , and sweat glands . in some conditions 
 , it may be associated with mental retardation . 
 we report a case of 10-year - old male child with ectrodactyly , syndactyly , ed , cleft lip / palate , hearing loss , and mental retardation .