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Epidermolysis bullosa simplex
Other Names for this Disease
- Epidermolysis bullosa intraepidermic
- Epidermolysa bullosa simplex with muscular dystrophy
- Epidermolysis bullosa
- Epidermolysis bullosa simplex with mottled pigmentation
- Epidermolysis bullosa simplex, Dowling-Meara type
- Epidermolysis bullosa simplex, generalized
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There is no cure for any of the types of epidermolysis bullosa simplex (EBS). Treatment of EBS typically involves supportive care to protect the skin from blistering, and the use of dressings that will not further damage the skin and will promote healing. Prevention of blisters may involve applying aluminum chloride to palms and soles to reduce blister formation; cyproheptadine may be used to reduce blistering in some individuals with the Dowling-Meara type of EBS. Keratolytics and softening agents for hyperkeratosis (thickening of the skin) of the palms and soles may prevent tissue from thickening and cracking. Prevention of secondary complications (such as infection of blisters) may involve treatment with topical and/or systemic antibiotics or silver-impregnated dressings or gels. Appropriate footwear and physical therapy may preserve ambulation in children with difficulty walking because of blistering and hyperkeratosis. Surveillance is recommended for infection and proper wound healing. Avoiding excessive heat, poorly fitting or coarse-textured clothing and footwear, and activities that traumatize the skin is typically recommended. Other treatments under investigation for epidermolysis bullosa include protein therapy and gene therapy.
Last updated: 1/19/2011
- Ellen G Pfendner and Anna L Bruckner. Epidemolysis Bullosa Simplex. GeneReviews. August 11, 2008; http://www.ncbi.nlm.nih.gov/books/NBK1369/. Accessed 1/19/2011.
- Kevin Berman. Episdermolysis Bullosa. MedlinePlus. October 31, 2008; http://www.nlm.nih.gov/medlineplus/ency/article/001457.htm. Accessed 1/19/2011.
- GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.
Clinical Trials & Research for this Disease
- ClinicalTrials.gov lists trials that are studying or have studied Epidermolysis bullosa simplex. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.