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Inherited epidermolysis bullosa: clinical and therapeutic aspects.

Boeira VL, Souza ES, Rocha Bde O, Oliveira PD, Oliveira Mde F, Rêgo VR, Follador I - An Bras Dermatol (2013 Mar-Apr)

Bottom Line: Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated.Diagnosis must be based on clinical and histopathological findings.To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.

View Article: PubMed Central - PubMed

Affiliation: Federal University of Bahia (UFBA), Salvador, BA, Brazil.

ABSTRACT
Inherited epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.

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Appearance of lesions in patients with dystrophic epidermolysis bullosa
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f03: Appearance of lesions in patients with dystrophic epidermolysis bullosa

Mentions: Dystrophic epidermolysis bullosa (DEB) is due to mutations in the gene encoding typeVII collagen, resulting in defective anchoring fibrils and consequent separation ofthe sub-basal lamina.11 Whenhealed, blisters give way to dystrophic lesions (Figure 3). Millium formation occurs due to damage in thehair follicles.19


Inherited epidermolysis bullosa: clinical and therapeutic aspects.

Boeira VL, Souza ES, Rocha Bde O, Oliveira PD, Oliveira Mde F, Rêgo VR, Follador I - An Bras Dermatol (2013 Mar-Apr)

Appearance of lesions in patients with dystrophic epidermolysis bullosa
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3750879&req=5

f03: Appearance of lesions in patients with dystrophic epidermolysis bullosa
Mentions: Dystrophic epidermolysis bullosa (DEB) is due to mutations in the gene encoding typeVII collagen, resulting in defective anchoring fibrils and consequent separation ofthe sub-basal lamina.11 Whenhealed, blisters give way to dystrophic lesions (Figure 3). Millium formation occurs due to damage in thehair follicles.19

Bottom Line: Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated.Diagnosis must be based on clinical and histopathological findings.To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.

View Article: PubMed Central - PubMed

Affiliation: Federal University of Bahia (UFBA), Salvador, BA, Brazil.

ABSTRACT
Inherited epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.

Show MeSH
Related in: MedlinePlus