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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CK 19 is found in the outermost layer of the outer root ar sheath, also denominated the basal layer of this sheath, this being more specific to the hair follicle Figure 3. In another variant, described by Pasini, there is also involvement of the oral mucosa and teeth, but blistering is more extensive and similar to papules on the trunk epidsrmolise lesions.

Histol Histopathol ; Diagnosis must be based on clinical and histopathological findings. The generalized atrophic benign HEB is a relatively mild subtype characterized by skin blisters present at birth. Although the disease is described as precociously fatal, some individuals with milder symptoms can survive during childhood. J Hand Surg Br.

CK have a characteristic distribution in each epithelium and its annexes. J Am Acad Dermatology. In recent years, it has been possible to explain the molecular genetics of some corneal dystrophies with mutations of CK present in that epithelium. The absence of effective therapeutic measures to control this disease shows the need for monitoring aiming to provide greater physical and psychological comfort. In a similar way, antivimentin antibodies are used in the diagnosis of mesenchymal tumors, however the great number of cytokeratins means that their use in the diagnosis of the epithelial neoplasias is much more widespread than in the case of the vimentin.


Progressive contracture of the hands and feet mitten deformities may develop in the first year of life and is seen primarily in the Hallopeau-Siemenes subtype of RDEB. Lesions should be cleaned epieermolise solutions of low toxicity, such as saline solution and water.

There may be chronic renal failure secondary to hydronephrosis, streptococcal glomerulonephritis, mesangial IgA disease and amyloidosis. Expert Rev Pharmacoecon Outcomes Res. Answer key Paraneoplastic cutaneous manifestations: Information for all epidermooise Basal cell carcinomas express basal CK 5 and 14, CK 17 and CK 19 Figure 5a pattern similar to that of the hair follicle, 13,14 and the possibility has been suggested that they originate from the outer root sheath, 15 which correlates with clinical findings, since this tumor is not found in palmar or plantar areas.

Their importance in the pathogenesis of several dermatoses is due to the specificity of the expression of CK according to the epithelium involved, for instance, the epidermis or the corneal epithelium, and also because they can be expressed only in certain sectors of the epithelia.

Which types of Herlitz JEB are associated with pyloric atresia?

Epidermólise bolhosa hereditária: aspectos clínicos e terapêuticos

In the presence of anemia, reversible telogen effluvium may occur. Large acquired nevocytic nevi induced by the Kobner phenomenon. Mutations in the keratin gene as a cause of Meesmann-Wilke corneal dystrophy and autossomal dominant skin cornification disorders.


Mucous membranes are often affected by erosions, resulting in stenosis. Rio Branco, 39 Type VII collagen is required for Ras activation in epidermal tumorigenesis. J Clin Psychol Med Settings. Cytokeratins as markers of are neoplasms with follicular differentiation: 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 sjmples of the dressing Biosynthetic cellulose Dressing epidermoolise of cellulose, water, 0.

Epidermólise Bolhosa by Leonor Sá Pinto on Prezi

Osteopenia and osteoporosis are common manifestations of which type of Herlitz JEB? The Weber-Cockaine subtype EBS-WC is characterized by mild to severe blistering and palmoplantar topography, and patients may concomitantly show hyperhidrosis.

Epidermolysis bullosa naevi reveal distinctive dermoscopic pattern. Occurrence of at least three of the following characteristics indicates the use of systemic antibiotics: Surgical intervention may improve hand functionality, although repeated procedures are necessary to maintain this functionality.

Am J Dermatopathol ; Medical management of epidermolysis bullosa: Avoiding trauma is essential to manage pain. Curr Opin Ophthalmol ; J Dermatol Sci ; Individuals with this type of JEB have a life expectancy similar to that of the general population. HL, Schumann H, et al. There is corneal, conjunctival, tracheobronchial, oral, pharyngeal, esophageal, rectal, and genitourinary mucosal involvement.