Abstract

Hereditary ectodermal dysplasia is a condition associated with deformation of one or more ectodermal derivatives which are typically inherited as a cross-linked recessive trait so that the frequency and severity of the condition is more pronounced in males than in females. The objective of this report is to present two female patients with hereditary ectodermal dysplasia, each of hypohydrotic and hydrotic type. These patients showed reduced number of teeth with structural changes which could be explained by X chromosome inactivation i.e., “Lyon” hypothesis, and also the present cases throws light on practical difficulties encountered in clinical diagnosis.

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