Epidermolysis bullosa simplex is one of a group of genetic conditions called epidermolysis bullosa that cause the skin to be very fragile and to blister easily. Epidermolysis bullosa (EB) is a group of genetic conditions that result in easy blistering of the skin and mucous membranes. Blisters occur with minor trauma or . Prevención de Ampollas. Como cargar al bebé: Evite levantar el bebé o niño por debajo de los brazos. En su lugar, ponga una mano debajo del trasero y la otra.
|Published (Last):||16 November 2014|
|PDF File Size:||4.46 Mb|
|ePub File Size:||9.43 Mb|
|Price:||Free* [*Free Regsitration Required]|
Physiopathologically, EB is caused by anomalous adhesion of the epithelial components. Gene therapy approaches for epidermolysis bullosa.
Types of Epidermolysis Bullosa
EBS usually is inherited in an autosomal dominant manner, meaning that a defective gene inherited from a parent is enough bullosa develop the condition. Ann Chir Main Memb Super.
Only comments written in English can be processed. EB can also be an autoimmune disease in which the body produces antibodies to the structural components of the epidermopisis. The severity of EB is generally dependent upon many factors including type, subtype, and inheritance pattern.
To have an autosomal recessive disorder, you inherit two mutated genes, one from each parent.
Epidermolysis bullosa – Wikipedia
Sign up to receive the latest in research, news, and events! Junctional epidermolysis bullosa medicine. Blistering tendency usually worsens in summertime or in warm weather. EB is classified into distinct subtypes depending on the location of blistering within the cutaneous dermal-epidermal basement membrane zone.
Journal of Investigative Dermatology. Aplasia cutis congenita Amniotic ximple syndrome Branchial cyst Cavernous venous malformation Accessory nail of the fifth toe Bronchogenic bulllsa Congenital cartilaginous rest of the neck Congenital hypertrophy of the lateral fold of the hallux Congenital lip pit Congenital malformations of the dermatoglyphs Congenital preauricular fistula Congenital smooth muscle hamartoma Cystic lymphatic malformation Median raphe cyst Melanotic neuroectodermal tumor of infancy Mongolian spot Nasolacrimal duct cyst Omphalomesenteric duct cyst Poland anomaly Rapidly involuting congenital hemangioma Rosenthal—Kloepfer syndrome Skin dimple Superficial lymphatic malformation Thyroglossal duct cyst Verrucous vascular malformation Birthmark.
Orphanet: Epidermolisis bullosa simple tipo Koebner
His mother’s pregnancy had been full term and uncomplicated. Retrieved 11 October This disease is characterised by blister formation within the lamina lucida of the basement membrane zone : After each extraction, the healing process was very rapid and the patient did not complain about any pain Figure 3B. Over mutations have been identified in eppidermolisis condition.
Health care resources for this disease Expert centres 92 Diagnostic tests 43 Patient organisations 42 Orphan drug s 7. The teeth had advanced white spot lesions, and there was deposition of dental plaque on all teeth Figure 5.
Bullous disorders of childhood. Junctional epidermolysis bullosa Junctional epidermolysis bullosa generally becomes apparent at birth and may be severe. Dental caries risk in hereditary epidermolysis bullosa. The treatment for patients with EB is multidisciplinary and, unfortunately, no specific therapeutic regimen can cure the disease 7,8.
Approximately , people are affected worldwide and no definitive treatments have yet been developed 8,7. The frequency of milia, mild atrophic scarring, focal plantar hyperkeratosis and nail dystrophy is intermediate between that of localized EBS and EBS-DM see these termsand extracutaneous findings, other than occasional intraoral blistering, are rare.
Kindler syndrome In this type of EB, blistering may occur at multiple levels within the basement membrane zone, or in skin layers beneath it. The restorative treatment using glass ionomer cement was accomplished within 5 weeks. Clinical management for epidermolysis bullosa dystrophica. However, her physical development was delayed and her weight and height were compatible with that expected for a 9 year-old child. The fingers of both hands had become fused.
In other projects Wikimedia Commons. Retrieved from ” https: Clinical evaluation of patients with epidermolysis bullosa: Although the clinical manifestations suggested EB diagnosis in both cases presented hereby, confirmation was granted by histopathologic study conducted at Lauro de Souza Lima Hospital, Bauru, SP, Brazil. Views Read Edit View history. How to cite this article. There are many genetic and symptomatic variations of EB, but all share the prominent symptom of extremely fragile skin that blisters and tears from minor friction or trauma.
The various types of epidermolysis bullosa are largely defined by which layer the blisters form in. Evite ropas con elasticos apretados, ziperes, y botones o broches asperos.
Epidermolysis bullosa ep-ih-dur-MOL-uh-sis buhl-LOE-sah is a group of rare diseases that cause epidrrmolisis, blistering skin. Dental plaque was controlled with professional prophylaxis and use of 0. These mutations, or errors in the genetic code, do not allow the body to either produce an essential protein or produce a working form of the protein thus resulting in extremely fragile skin.
Epidermolysis bullosa Dpidermolisis comprises a group of genetically determined skin fragility disorders characterized by blistering of the skin and mucosa following mild mechanical trauma 18,20, Moreover, regular dental evaluation and treatment is imperative and should be instituted as early as possible 9,15similarly to what has been done for the patient of Case 2.
During the application of the local simlle solution, blister formation occurred. Two case reports with year follow-up.
Special dental concerns involve the use of soft toothbrushes and irrigation techniques.