Epidermolytic Keratosis is also referred to as bullous Congenital ichthyosiform. It is rare autosomal congenital infection of Keratosis and it is common in new borns. The infection is followed by a lifetime skin disorder . Epidermolytic keratosis is divided into 2 classes and these are ; the type 1 keratins{ these are acidic in nature}, and the type 2 keratinic actions which are the normal keratosis that is more prevalent on the skin. Keratins often form intermediate filaments especially when there is the presence of both type 1 and type 2 keratins .These filaments provide the adequate supports for keratinocytes .
Epidermolytic keratosis results from the mutation caused to the genes controlling keratin production. Clinical blistering as well as skin cell collapse are some of the problems associated with epidermolytic keratosis. At the unset of the blistering problems, the skin becomes thickened because it wants to protect itself from the blistering. Epidermolytic keratosis is a rare occurring skin disorder as it is believe to occur in 1 out of every 200,000 – 300,000 in the United States alone.
The mortalities and morbidity levels of epidermolytic keratosis include; sepsis, electrolyte imbalances, and recurrent infections. All these are also present even at the neo-natal periods. It is also believed that Epidermolytic infections does not have preference for any sex or race but it is a problem that starts especially at birth.
Erythema and blistering are some of the first noticeable symptoms of epidermolytic keratosis. These are the first symptoms that appear especially shortly after the child has been given birth to. These symptoms often ameliorate in some patients after some times. Most people who suffer from epidermolytic keratosis often inherit it and such people do have a long history of the infection. More than half of the cases recorded often result from sporadic mutations in the genes regulating keratin production levels in the body.
The growing epidermolytic keratosis infections often present some superficial blisters which tend to rupture and leave some raw denuded areas on the skin. Though, Epidermolytic keratosis often becomes more prominent in the third month, however some symptoms such as thickening and scaling of the skin often show up in the first month of the birth of the child. As the individual ages, the blisteing and thickening of the skin becomes more pronounced and the scaling becomes much more pronounced as the skin disorder worsens.
Epidermolytic keratosis is very disfiguring and embarrassing in nature.
