Ectodermal Dysplasia



Introduction

There are two groups of ectodermal dysplasia can be distinguished. In the first group, patients with disorders are at least two different ectodermal structures. In the second group only one of the above disorders is found, but in the context of another ectodermal disorder such as the ears, lips or sole of the foot (Bonilla 1997, 657-65). Ectodermal Dysplasia is a set of diseases that affect the skin, hair, and the teeth. Ectodermal dysplasia (ED) refers to a varied group of disorders that is mainly distinguished by developmental dystrophies of ectodermal structures such as hypotrichosis of the onychodysplasia, hypohidrosis, anodontia or hypodontia. Approximately 160 of ectodermal dysplasia patients inherited genetically and clinically distinct (Itthagarun 1997, 595-602).

Many people with this disease are observed with abnormal structure of the teeth, as well as defects in the structure of the skull and face (thick lips, and abnormally large chin). Human skin with this disease is characterized be very thin and dry, with impaired pigmentation. However, the skin around the eyes can be dark; can be wrinkles around the eyes in infants and children with this disease, except that there is a lack of glands in the gastro-intestinal and respiratory tract. In some cases, a weakened immune system, so the person becomes very vulnerable and susceptible to infections, diseases, allergic reactions. Develop chronic inflammation of the nasal mucosa, skin rash (eczema), may also be other symptoms. The ectodermal dysplasia (ED) is a heterogeneous group of hereditary defects, the malformation (dysplasia) of those structures give rise to the outer germ layer (ectoderm arise), for example, hair, nails, teeth and skin including the sweat glands.

q1. The real question: What are other physical signs and symptoms that Ralph would be likely to have as a result of Ectodermal Dysplasia?

The diagnosis of ectodermal dysplasia is carried through to clinical signs (abnormal face, dental abnormalities, unexplained fever, sweating, etc.). Ectodermal dysplasia anidrotique also called Christ-Siemens-Touraine, which is characterized by congenital absence of sweat glands resulting in impairment of adaptation to heat and accompanied by a complete or partial absence of teeth (anodontia) (Itthagarun 1997, 595-602). It also results in;

Thick nails

Eyelashes for

Excessive sweating

Ralph is also most likely to go through an abnormatlity of the hair follicles. The scalp along with the body hair is going to be sparse, thin as well as lighter in color. The growth of the hair will be sporadic by nature. But the hari is going to be fragile and either be curly or twisted.

The hypohidrotic ectodermal dysplasia is characterized by a soft, dry and thin skin, often associated with palmoplantar keratosis. It caused increased pigmentation around the mouth and eyes, which can be well be observed. The skin is also going to have red and brown pigmentation. Rash as well as infection is going to be prominent in the soles as well as the palms. Ralph must take care before it further cracks, bleeds and infects.

The finger as well as the toenails is going to be thick, discolored and abnormally shaped along with ...