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Epidermolysis Bullosa

A group of chronic skin disorders in which fluid-filled blisters form on the skin and mucosa (the moist, inner lining of some organs and body cavities).

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About Epidermolysis Bullosa

Epidermolysis bullosa is a group of blistering skin conditions. The skin is so fragile in people with epidermolysis bullosa that even minor rubbing may cause blistering. At times, the person with epidermolysis bullosa may not be aware of rubbing or injuring the skin even though blisters develop. In severe epidermolysis bullosa, 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 genitals.

The skin has an outer layer called the epidermis and an underlying layer called the dermis. The place where the two layers meet is called the basement membrane zone....Read more about Epidermolysis Bullosa NIH - National Institute of Arthritis and Musculoskeletal and Skin Diseases

What works? Research summarized

Evidence reviews

Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita (rare autoimmune blistering diseases of the skin, eyes and mouth)

Mucous membrane pemphigoid and epidermolysis bullosa acquisita are rare autoimmune blistering diseases of the skin and mucous membranes (eyes and mouth). They can result in scarring, which may lead to disabling and life threatening complications. Treatments include corticosteroids, mycophenolate mofetil and cyclophosphamide to suppress the immune system, and less toxic drugs such as antibiotics. These diseases often progress despite treatment. There is some evidence that mucous membrane pemphigoid involving the eyes may respond better to treatment with cyclophosphamide combined with corticosteroids, compared to treatment with corticosteroids alone. Cyclophosphamide is, however, associated with potentially severe adverse effects. Dapsone may help moderate disease. More research is needed to identify the most effective treatment options.There is not enough reliable evidence about treatments for the rare blistering diseases, mucous membrane pemphigoid and epidermolysis bullosa acquisita.

A systematic review of randomized controlled trials of treatments for inherited forms of epidermolysis bullosa

BACKGROUND: Many interventions have been described for inherited epidermolysis bullosa (EB), but it is unclear which are beneficial.

Behavioral Counseling to Prevent Skin Cancer: Systematic Evidence Review to Update the 2003 U.S. Preventive Services Task Force Recommendation [Internet]

We conducted this systematic evidence review of five key questions to assist the U.S. Preventive Services Task Force (USPSTF) in updating its 2003 recommendation on behavioral counseling to prevent skin cancer (melanoma, basal cell carcinoma, and squamous cell carcinoma).

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Summaries for consumers

Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita (rare autoimmune blistering diseases of the skin, eyes and mouth)

Mucous membrane pemphigoid and epidermolysis bullosa acquisita are rare autoimmune blistering diseases of the skin and mucous membranes (eyes and mouth). They can result in scarring, which may lead to disabling and life threatening complications. Treatments include corticosteroids, mycophenolate mofetil and cyclophosphamide to suppress the immune system, and less toxic drugs such as antibiotics. These diseases often progress despite treatment. There is some evidence that mucous membrane pemphigoid involving the eyes may respond better to treatment with cyclophosphamide combined with corticosteroids, compared to treatment with corticosteroids alone. Cyclophosphamide is, however, associated with potentially severe adverse effects. Dapsone may help moderate disease. More research is needed to identify the most effective treatment options.There is not enough reliable evidence about treatments for the rare blistering diseases, mucous membrane pemphigoid and epidermolysis bullosa acquisita.

Terms to know

Blister
A fluid-filled sac in the outer layer of skin. It can be caused by rubbing, heat, or diseases of the skin.
Dermatologist
A doctor who has special training to diagnose and treat skin problems.
Dermis
The inner layer of the two main layers of the skin. The dermis has connective tissue, blood vessels, oil and sweat glands, nerves, hair follicles, and other structures. It is made up of a thin upper layer called the papillary dermis, and a thick lower layer called the reticular dermis.
Epidermis
The outer layer of the two main layers of the skin.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Mucosa (Mucous Membranes)
The moist, inner lining of some organs and body cavities (such as the nose, mouth, lungs, and stomach). Glands in the mucosa make mucus (a thick, slippery fluid). Also called mucous membrane.
Skin
The outer covering of the body that protects it from the environment.

More about Epidermolysis Bullosa

Photo of a child

Also called: Acantholysis bullosa, Acanthosis bullosa, Dermatitis bullosa hereditaria, Fox disease, Keratolysis bullosa hereditaria, EB

Other terms to know: See all 7
Blister, Dermatologist, Dermis

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