Epidermolysis Bullosa - Causes, Symptoms & Treatment

Epidermolysis bullosa (EB) is a group of inherited bullous disorders characterized by blister formation in response to mechanical traumal. EB is a group of blistering skin conditions. The skin is so fragile in people with EB that even minor rubbing may cause blistering. Skin is so fragile in people with EB that even minor rubbing may cause blistering. At times, the person with EB may not be aware of rubbing or injuring the skin even though blisters develop. 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. Epidermolysis Bullosa (EB) is classified into 3 major categories, including (1) EB simplex (EBS; intraepidermal skin separation), (2) junctional EB (JEB; skin separation in lamina lucida or central BMZ), and (3) dystrophic EB (DEB; sublamina densa BMZ separation.

Epidermolysis Bullosa blisters form by cytolysis in the basal or spinous layers of the epidermis, and keratinocytes often have abnormal density and organization of keratin filaments. Most people with EB have inherited the condition through faulty genes they receive from one or both parents. Genes are located in the body's cells and determine inherited traits passed from parent to child. They also govern every body function, such as the formation of proteins in the skin. More than 10 genes are known to underlie the different forms of EB. Genes are located on chromosomes, which are structures in each cell's nucleus. In junctional EB (JEB), the epidermis separates from the basal lamina, forming a blister cavity in the plane of the lamina lucida where hemidesmosome structure and density are frequently diminished.

Dystrophic epidermolysis (DEB), the basal lamina remains attached to the epidermis, but the blister cavity forms beneath the lamina densa of dermoepidermal junction, and anchoring fibrils may appear abnormal, reduced in number, or altogether absent Blisters form by cytolysis in the epidermis or basement membrane zone of the skin.

Causes of Epidermolysis Bullosa

Common causes of Epidermolysis Bullosa

• Keratin filaments.
• Hemidesmosomes.
• Anchoring filaments.
• Anchoring fibrils.
• Genetics disorder ( autosomal dominant disorder and autosomal recessive disorder).

Symptoms of Epidermolysis Bullosa

Common Symptoms of Epidermolysis Bullosa

• Skin blisters.
• Nail loss or deformed nails.
• Hoarse cry.
• Cough.
• Dental abnormalities.
• Alopecia (hair loss).
• Milia (tiny white bumps or pimples).

Treatment of Epidermolysis Bullosa

Common Treatment of Epidermolysis Bullosa

• Use of steroids for epidermolysis bullosa.
• Avoid getting too hot by keeping rooms at an even temperature
• Apply lotion to the skin to reduce friction and keep the skin moist
• Wear soft clothing
• Use sheepskin on car seats and other hard surfaces
• Wear mittens at bedtime to help prevent scratching.
• Regular skin care and dressing
• Videofluoroscopy and/or barium swallow.

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