Epidermolysis Bullosa
Epidermolysis bullosa (EB) is a gathering of inherited bullous disorders classified by lesion formation in respond to mechanical traumal. EB is a group of severe skin conditions. The skin is so delicate in people with EB that even minor rubbing can cause blistering. Skin is so fragile in people with EB that even minor scratch can cause blistering. At times, the person with EB may not be kmowledgeable of rubbing or injuring the skin even though blisters form.
In severe EB, blisters are not enclosed to the outer skin. They may occur in the body, in places like the linings of the mouth, esophagus, stomach, intestines, upper airway, bladder, and the genitals. Epidermolysis Bullosa (EB) is categorised 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 lesions forms by cytolysis in the basal or spinous layers of the epidermis, and keratinocytes usually have irregular solidity and organization of keratin filaments. Most people with EB have familial the condition from defective genes they get from one or both parents. Genes are installed in the body’s cells and determine inherited traits flowa from parent to child. They also manage every body function, such as the production of proteins in the skin. More than 10 genes are located in different forms of EB. Genes are located on chromosomes, which are structures in each cell’s nucleus. In junctional EB (JEB), the epidermis distinct from the basal lamina, forming a lesion cavity in the plane of the lamina lucida where hemidesmosome form and density are frequently belittle.
Dystrophic epidermolysis (DEB), the basal lamina remains joined to the epidermis, but the lesion cavity forms beneath the lamina densa of dermoepidermal junction, and anchoring fibrils can emerge abnormal, lessened in number, or entirely absent. Blisters form by cytolysis in the epidermis or basement membrane area 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.
Filed under: Skin Disorders