21%) had conical shaped teeth; 13 cases (68.42%) had high arched palate; 19 cases (100.00%) had thin alveolar bone; 4 cases (21.05%) had taurodontism and in 1 case (05.26%) cleft lip and cleft full article palate [Table 3]. The number of teeth present in all the cases ranged from 0 to 19 [Graph 4]. Table 3 Oral manifestations observed in cases of ectodermal dysplasia Graph 4 Number of teeth present DISCUSSION ED is a rare congenital hereditary disorder that occurs as a consequence of disturbances in the ectoderm of the developing embryo. ED is usually transmitted as an X-linked recessive trait, in which gene is carried by the females and manifested in males.[1,18] Hence men are more frequently and severely affected.[16] The heterozygote usually show minor defects.

In the present study, more number of males were affected then females with a ratio of 1.7:1. Our findings did not match with the findings of Yavuz Izzet et al.,[1] wherein the males and females were equally affected. We also noted that, hypohydrotic type was more common then hydrotic type. This finding was similar to the findings of Tauseef Kamran et al.[16] and Giansanti et al.[19] The Latin word consanguineous means ��of common blood��. A consanguineous marriage is one in which two individuals related by blood, get married and have children. Consanguineous persons have at least one common ancestor in the preceding few generations. The risk of birth defects is higher in consanguineous marriages because there is a greater chance of two related individuals sharing a common harmful gene and both of them passing it on to the child.

The risk and type of birth defects in consanguineous marriages varies according to how closely the couple is related and thus the chance of inheriting a recessive disorder is increased. Most consanguineous couples can have normal healthy children.[20] It is significant to note that the marriage history of parents of all the cases revealed that history of consanguineous marriages were more than nonconsanguineous marriages. This finding when correlated with the number of offspring affected with ED, it was distinctly observed that a consanguineous marriage was directly proportional to ED. Various database search could not reveal studies on this variable. The result of our analysis directs towards the need of establishing the prevalence and occurrence of ED in children born to consanguineous parents.

ED manifests with numerous clinical Cilengitide features, both general and oral. The characteristic signs of ED include sparse hair, anodontia or hypodontia and inability to sweat due to lack of sweat glands.[4] All the cases in the present study were diagnosed on the basis of these features. We noticed that partial anodontia was seen in majority of the patients and complete anodontia in one case. Multiple missing teeth were seen more in the mandible and both the deciduous and permanent dentition were involved.