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.
|Published (Last):||11 April 2010|
|PDF File Size:||5.22 Mb|
|ePub File Size:||16.66 Mb|
|Price:||Free* [*Free Regsitration Required]|
Epidermolose type of inheritance is observed in DEB? In the dominant subtype DDEB clinical manifestations usually occur at birth or during childhood, with generalized blistering.
Molecular Genetics of Epidermolysis Bullosa
In the epidermis, for instance, they can have a basal expression Figure 2 – CK 5 and 14 – or suprabasal – CK 1 and These lesions may recur frequently even with epiidermolise surgical excision.
Na EBD a clivagem ocorre: J Clin Bolhoa ; A new clinical perspective of corneal dystrophies through molecular genetics. Although Herlitz JEB is a quite rare dermatological disorder, its impact on the lives of patients and their families is immeasurable, bringing great physical and emotional suffering and several limitations to the individual.
Two cases of atypical melanocytic lesions in recessive dystrophic epidermolysis bullosa infants. 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.
Molecular genetics of heritable blistering disorders. In older children, the use of special shoes and foam in the knee to prevent blistering is recommended. With respect to the treatment of lesions, blister puncturing to prevent dissemination and use of sterile dressings are recommended. The basic principle underlying the care of patients with Herlitz JEB is to prevent blistering with meticulous skin protection and prevention of infections through wound care.
Both can occur in childhood, causing scarring and progressive visual impairment, if not treated. Appearance of lesions in patients with junctional epidermolysis bullosa. Acta Derm Venereol ; There is no instrument available for clinical outcome research measurements.
The experimental induction 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.
In epieermolise of DEB, there is no predilection bolhpsa photoexposed areas. Hydrogel dressings, contact layers and biosynthetic cellulose can also be used. They have a perforated surface, allowing the passage of exudate into the dressing If there is significant bleeding or exudate, the dressing will adhere Contact layers Inert protection material, which allows the non-traumatic removal of the dressing Biosynthetic cellulose Dressing composed of cellulose, water, 0.
Epidermólise bolhosa hereditária: aspectos clínicos e terapêuticos
Mitsuhashi Y, Hashimoto I. Hand deformities should be prevented with appropriate dressings involving all fingers at night. They are generally associated with cytolysis, since the cell collapses as a result of alterations in bolhhosa cytoskeleton.
Plastic filmthat prevents adherence of the dressing to the wound epudermolise. The antiadherent and absorbent characteristics of dressings were crucial in the choice of materials, for this is a disease that evolves with bullous lesions, extensive exulcerated wounds, high chances of infection and scarification. The activity of the lesions is compounded by increasing temperature, healing with a distinctive atrophic appearance.
The intermediate filaments are capable of autopolymerization, forming a cytoplasmatic network responsible for the mechanical strength of the cells, and in the case of the epithelia, are important against eventual simple trauma.
The risk of melanoma and BCC in other subtypes is comparable to that of the general population. DEB may be associated with autosomal recessive or dominant inheritance. Lesions should be cleaned with solutions of low toxicity, such as saline solution and water.
Cytokeratins CK belong to the intermediate filament group and are expressed in epithelial cells. Localized or diffuse alopecia can be observed in Herlitz JEB. Translated from the original published in the Journal of the American Academy of Dermatology; EBS is subdivided as follows: Dressings with borders can sometimes be very adhesive and should be used with caution.