16 mar. Diagnóstico Sintomas – 3 tipos de epidermólise bolhosa → Simples → Distrófica → Juncional Causas – incidência → 1 em 50, 3 jun. Epidermólise Bolhosa (EB) O que é? Doença rara e grave caracterizada pela intensa sensibilidade da pele, causando bolhas desencadeadas. Epidermólise bolhosa hereditária: aspectos clínicos e terapêuticos. Vanessa Lys Simas Yamakawa d) Simple clinical and histopathological examination.

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Epidermólise bolhosa hereditária: aspectos clínicos e terapêuticos

Patients with esophageal stenosis must undergo dilation to maintain an adequate intake of nutrients orally. They have a perforated surface, allowing the passage of exudate into the dressing.

Br J Dermatol ; Watch out for infections. They are currently divided into two groups: Histol Histopathol ; A de novo glycine substitution mutation in the collagenous domain of COL7A1 in dominant dystrophic epidermolysis bullosa.

Inherited epidermolysis bullosa: clinical and therapeutic aspects

The alteration of the basal layer by mutation, whether CK 5 or 14, leads to the degeneration of the basal layer Figure 6with the fragility and formation of blisters, characteristic of epidermolysis bullosa simplex EBS 19,20 the first illness in which the mutation of a CK. Inert protection material, which allows the non-traumatic removal of the dressing. J Cell Sci ; Although uncommon in simlles with RDEB-HS, there may be dilated cardiomyopathy, possibly fatal, especially when there is associated renal failure.

The “boxing glove hands” can be temporarily improved with surgical procedures. Which form of DEB is associated with generalized blistering, predominantly in acral surface, leading to pseudosyndactyly of the hands boxing glove hands and feet?


Appearance of lesions in patients with junctional epidermolysis bullosa. Pediatr Dermatol ; 32 1: Correction of junctional epidermolysis bullosa by transplantation of genetically modified epidermal stem cells. The accumulation of lymph and moisture in the surface increases bacterial growth.

Curr Opin Ophthalmol ; Allow large quantities of fluid and drained material from wounds to be absorbed. A epiderme expressa citoqueratinas diferentes na camada basal e nas camadas suprabasais. Calcium alginate dressings release calcium ions which help to stop bleeding.

Mol Biol Cell ; It is seen almost exclusively in some subtypes of JEB and can occur in the first year of life.

The experimental epldermolise of CK 6 has already been demonstrated, when the epidermis is stimulated by cytokines, such as interferon gamma, epithelial growth factor EGF and tumor necrosis factor TNFas well as by ultraviolet radiation, and the latter also induces CK 19, thus illustrating the dynamic aspects of CK expression.

These lesions may recur frequently even with aggressive surgical excision. Skin lesions usually disappear without scarring. Eruptive melanocytic nevi after Stevens-Johnson syndrome.

Questions and Answers to Questions. The severity of skin lesions does not necessarily correlate with the degree of muscular dystrophy.

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Curative options for each type of lesion are shown in table 3 ; the properties of each category of dressings are shown in table 4. CQ 19 e Hiram Larangeira de Almeida Jr.

Studies of allogeneic keratinocyte grafting have shown improvement of unhealed wounds in patients with Herlitz JEB, reducing trauma and promoting reepithelialization.


The milder forms of EBS present with blisters that are usually caused by an identifiable traumatic event. The application of monoclonal antibodies markers of CK enables an investigation into the origin of tumors and their differential diagnosis. Enamel hypoplasia, which facilitates the formation of cavities and tooth loss, is a pathognomonic characteristic of all types of JEB, while microstomia and ankyloglossia are typical of RDEB.

N Engl J Med ; Likewise, the trichokeratins are also divided into two groups, denominated according to convention as group I from hHa1 to hHa8 human Hair acidic and group II from hHb1 to hHb6 human Hair basicaccording to their location in the two-dimensional electrophoresis. The use of cultured allogenic keratinocyte grafting in a patient with epidermolysis bullosa simplex. In most patients, even with correction of pyloric atresia, prognosis is unfavorable, given the extent of systemic involvement.

However, the mechanism and the risk and benefits of this aggressive treatment in the long term need to be better understood. Compound heterozygosity for nonsense and missense mutations in the LAMB3 gene in nonlethal junctional epidermolysis bullosa. Am J Clin Dermatol ; 4: Produzir a haste do cabelo. Aumailley M, Krieg T.

Infants require greater care and control of the environment around them to prevent trauma.