Epidermólise bolhosa distrófica recessiva mitis: relato de caso clínico. Article ( PDF Available) in Anais Brasileiros de Dermatologia 80(5) · October with Início · Epidermólise Bolhosa · O que é a EB? Cuidados Básicos · Info para nova realidade num mundo cheio de desafios. O que é a Epidermólise Bolhosa?. A epidermólise bolhosa hereditária (EBH) compreende um grupo heterogêneo de desordens genéticas que têm em comum a fragilidade cutânea e, em alguns .

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Muscular dystrophy beginning in adulthood is typical of EBS with muscular dystrophy. J Pediatr Gastroenterol Nutr.

Epidermólise bolhosa hereditária: aspectos clínicos e terapêuticos

Genital lentigines and melanocytic nevi with superimposed lichen sclerosus: Epidermolysis bullosa, Epidermolysis bullosa dystrophica, Epidermolysis bullosa, junctional, Epidermolysis bullosa simplex. It usually shows clinical manifestations similar to those of other inherited forms of dystrophic EB. As its name suggests, EBS with pyloric atresia presents with pyloric atresia at birth and, usually, blistering is widespread.

The most significant complication is partial or complete occlusion of the airways, usually resulting from stenosis of the vocal cords, which can quickly lead to death. 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.


Long-term follow-up of patients with Herlitz-type junctional Epidermolysis bullosa. With extensive bare areas bolhosx areas of crusting, strict care must be taken. EBS is epiddrmolise as follows: The severity of skin lesions does not necessarily epidermokise with the degree of muscular dystrophy.

Patients with severe RDEB who survive childhood have a significant risk of developing aggressive squamous cell carcinoma in areas of chronic lesions. In most patients, even with correction of pyloric atresia, prognosis is unfavorable, given the extent of systemic involvement.

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

Plastic filmthat prevents adherence of the dressing to the wound surface. Herlitz junctional epidermolysis bullosa: Services on Demand Journal.

These lesions may recur frequently even with aggressive surgical excision. Nat Cell Biol ; 3: Gene therapy for genetic skin disease. Assinale a alternativa correta.

Epidermólise Bolhosa by Leonor Sá Pinto on Prezi

Malabsorptive syndrome may be secondary to denudation of the small bowel mucosa. The formation of recurring vesicles along the urethra, in the ureterovesical junction and ureters can generate obstructive processes culminating with hydronephrosis.

Junctional epidermolysis bullosa Junctional epidermolysis bullosa JEB is an autosomal recessive disorder characterized by separation of the lamina lucida in the dermo-epidermal junction.

Kindler syndrome is not associated with alopecia. Prenat Diagn ; It is correct to state the following about anemia in patients with Herlitz JEB: New local therapeutic strategies include the use of biological or skin-like dressings. Corrective gene transfer of keratinocytes from patients with junctional epidermolysis bullosa restores assembly of hemidesmossomes in reconstructed epithelia. Patients with esophageal stenosis must undergo dilation to maintain an adequate intake of nutrients orally.


Hydrogel dressings, contact layers and biosynthetic cellulose can also be used. Please review our privacy policy.

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Exp Dermatol ; 9: 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. 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.

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.

Self-adhesive dressing is a good choice to keep the areas covered.