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Epidermolysis Bullosa - Symptoms and Treatment

EB is a group of blistering skin conditions. These may occur anywhere on the body but most commonly appear at sites of friction and minor trauma such as the feet and hands. Blisters may also occur on internal organs, such as the oesophagus, stomach and respiratory tract, without any apparent friction. The inherited form of EB has three major subtypes-EB simplex (EBS), junctional EB (JEB), and dystrophic EB (DEB)-based on whether blisters arise in the epidermis or in the middle (i.e., the lamina lucida) or undersurface of the lowermost portion (i.e., the lamina densa) of the dermoepidermal junction, respectively. By currently accepted convention, individual subtypes of inherited EB are further defined by their model of inheritance, ultrastructural site of skin cleavage, appearance and number of specific structures in keratinocytes and the adjacent dermoepidermal junction, and the presence or absence of specific cutaneous and extracutaneous manifestations. The epidermis is the outermost layer of skin and "lysis" refers to a breakdown of the structure within this layer. Bullosa means blister. EB, therefore, refers to a condition that causes the breakdown and blistering of the outer layer of skin. People with EB have extremely delicate skin, and children with the illness have been described as "Butterfly Children", as their skin is considered to be as fragile as a butterfly's wing. EB can take over 20 different forms, each of which has characteristic symptoms. Some forms of EB are quite mild, but others can be more severe and incapacitating.

Epidermolysis bullosa is a group of inherited disorders in which skin blisters develop in response to minor injury. In severe EB, blisters are not confined to the outer skin. They may develop inside the body, in such places as the linings of the mouth, esophagus, stomach, intestines, upper airway, bladder, and the genitals. Every year it affects less than 10,000 children and adults in the United States. According to the latest figures, most of these patients suffer from the simplex form of EB, about 600 have the Junctional form, 600 have RDEB, 840 with DDEB and 320 with some unclassified form of EB.

Causes of Epidermolysis Bullosa

Common Causes of Epidermolysis Bullosa :

  • Factors such as chafing, rubbing of the skin or even increase ion room temperature may cause blisters to form.
  • Pidermolysis Bullosa generally caused by skin's structural abnormalities.Mostly Epidermolysis bullosa results due to the molecule abnormality.
  • Epidermolysis bullosa (EB) may be a result of a dominant genetic abnormalit. Change in the DNA in the genes that dictate the way our bodies produce skin leads to the disease.
  • Some infants may have large blisters at birth. Some develops shortly after birth.

Symptoms of Epidermolysis Bullosa

Some common Symptoms of Epidermolysis Bullosa :

  • Nail loss or deformed nails.
  • Blistering around the eyes and nose.
  • Blistering present at birth.
  • Alopecia (hair loss).
  • A hoarse cry, cough, or other respiratory difficulties.
  • Dental abnormalities such as tooth decay.

Treatment of Epidermolysis Bullosa

  • Treatment includes the application of appropriate non-adherent dressings to aid wound healing and prevent further damage and infections.
  • Good dental hygiene is very important, including regular dental visits.
  • To prevent infection, excellent skin care is required, especially if any blistered areas become crusted or denuded.
  • Proper nutrition is also important. When skin injury is extensive, increased calorie and protein intake may be necessary to help recovery.








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