Ectodermal dysplasia Ectoderm

dental abnormalities in 5-year-old girl north sweden family suffered various symptoms of autosomal dominant hypohidrotic ectodermal dysplasia (hed) a) intraoral view. note upper incisors have been restored composite material disguise original conical shape. b) orthopantomogram showing absence of ten primary , eleven permanent teeth in jaws of same individual.

hypohidrotic ectodermal dysplasia (hed) common subtype of disease. clinical cases of patients condition displayed range of symptoms. 1 of common abnormalities of hed hypohidrosis, or inability sweat, can attributed dysfunctional sweat glands. aspect can dangerous in warm climates patient potentially suffer hyperthermia. facial malformations related hed such disfigured or absent teeth, wrinkled skin around eyes, misshaped nose along scarce , thin hair. skin problems, eczema have been observed in cases. typically follows x-linked recessive pattern of inheritance of eda genes. disease typically affects males because have 1 x chromosome, meaning 1 copy of mutated gene enough cause abnormal development. females affected, both x chromosomes need carry gene mutation. if female has mutated version of gene on 1 x chromosome, considered carrier of disease.