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):||4 January 2014|
|PDF File Size:||6.25 Mb|
|ePub File Size:||9.56 Mb|
|Price:||Free* [*Free Regsitration Required]|
Produzir a haste do cabelo. Mortality of bullous skin disorders from through in the United States. An update on keratin mutations in epidermolysis bullosa simplex Dowling-Meara in press.
There may be chronic renal failure secondary to hydronephrosis, streptococcal peidermolise, mesangial IgA disease and amyloidosis. Generally, KS is associated with disruption of the basement membrane and abnormal deposition of type VII collagen both in regions with active lesions and in lesion-free areas.
Immunohistochemical examination shows that blistering occurs in the lamina lucida. In some patients with cryptogenic cirrhosis, which occurs without viral hepatitis, alcoholism or any other known cause, mutations have been described in CK 8 and Arch Dermatol Res ; An example of a new gene therapy strategy was reported in a study conducted in Italy with a 36year-old patient with JEB.
Large atypical melanocytic nevi in recessive dystrophic epidermolysis bullosa: 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.
For scarified wounds, hydrogel dressings, biosynthetic cellulose and hydrocolloid are the best choices, given their debriding action.
Inherited epidermolysis bullosa: clinical and therapeutic aspects
Kindler syndrome is not associated with alopecia. The catalog of human hair keratins I. Mucous membranes are often affected by erosions, resulting in stenosis. J Eu Acad Dermatol Venereol ; It is correct to state the following about anemia in epidremolise with Herlitz JEB: In the last decade, several mutations were described in CK genes, which lead to structural changes in its molecule.
Br J Dermatol ; Topical or systemic antibiotics may be used for short periods following established criteria to avoid bacterial resistance and sensitization.
However, the mechanism and the risk and benefits of this aggressive treatment in the long term need to be better understood. Patients with Herlitz JEB subtypes showing a well-known risk of extra-cutaneous complications require careful monitoring and appropriate intervention medical, surgical, dental, nutritional and psychological before the tissues involved become severely epidermolose. In the presence of anemia, reversible telogen effluvium may occur.
Factors affecting quality of life in epidermolysis bullosa. Generalized dystrophic epidermolysis bullosa: Thin layer of absorbent cotton fibers that are placed between layers of polyethylene terephthalate with perforations and sealed borders. There is gradual alopecia in areas of frictional trauma and blistering in patients with DEB.
Portal Regional da BVS
They are generally associated with cytolysis, since the cell collapses as a result of alterations in the cytoskeleton. In DDEB many studies are being conducted focusing on ways that negatively regulate the dominant negative gene, or, alternatively, that bolhsoa its presence with up regulation of other genes whose products may offer, even if partially, better skin structural stability, replacing the effect of the underlying mutation.
Pulkkinen L, Uitto J. Junctional epidermolysis bullosa gravis Herlitz: The follicular section above the sebaceous gland expresses the same CK as the epidermis. Large acquired nevocytic nevi induced by the Kobner phenomenon. Which criteria are taken into consideration prior to bolgosa use of topical antibiotic therapy in patients with Herlitz JEB? Herlitz junctional epidermolysis bullosa: Prenatal diagnosis of dominant dystrophic epidermolysis bullosa, by COL7A1 molecular analysis.
Psychiatric symptoms are observed in all types of Herlitz JEB. Pilot testing revealed that combined see below and subscores were able to differentiate between EB subtypes and degrees of clinical severity EB simplex The clinical spectrum of epidermolysis bullosa simplex.
Other characteristics such as extension and location of lesions, frequency of dressing changes, cost, and economic status of the patient should also be considered.
Exogenous agents such as glucocorticoids and penicillamine contribute to impaired wound healing. J Dermatol Sci ; Novel COL7A1 mutations in dystrophic forms of epidermolysis bullosa.
Epidermólise Bolhosa (EB) by Sara Fontes on Prezi
Debridement becomes less necessary when wounds are treated in epidermooise early stages with appropriate coverings; minimal debridement can be done during the patient’s bath. Pulkkinen L, Uitto J. In the simple epithelium, found in the liver, pancreas and intestine, illnesses have also been verified associated to mutations, involving the cytoskeletons, described recently. Kindler syndrome KS is an autosomal recessive genodermatosis that can clinically simulate all three classic types of inherited EB.
Na EBD a clivagem ocorre: Novel K5 and K14 mutations in German patients with the Weber-Cockayne variant of epidermolysis bullosa simplex.