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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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Representation of the proteins affected in different types of inheritedepidermolysis bullosa
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f01: Representation of the proteins affected in different types of inheritedepidermolysis bullosa

Mentions: Inherited epidermolysis bullosa (EB) is a group of genetically transmitted skindisorders characterized by spontaneous blistering or blistering caused by minortrauma.1,2 There are three classic types of inherited EB (simplex,junctional and dystrophic). They are differentiated by the level of blister cleavage andsubdivided according to the pattern of genetic inheritance, morphology/topography oflesions and genetic mutation involved. After the Third International Consensus onDiagnosis and Classification of Inherited EB, there was the addition of a fourth entityto the group of inherited EB - Kindler syndrome (KS) previously considered aphotosensitive poikiloderma.3,4 Currently, over 30 phenotypiccally andgenetically distinct nosological entities have been described (Table 1).1,3Figure 1 illustrates the key molecules involved inthe pathogenesis of EB.


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)

Representation of the proteins affected in different types of inheritedepidermolysis bullosa
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f01: Representation of the proteins affected in different types of inheritedepidermolysis bullosa
Mentions: Inherited epidermolysis bullosa (EB) is a group of genetically transmitted skindisorders characterized by spontaneous blistering or blistering caused by minortrauma.1,2 There are three classic types of inherited EB (simplex,junctional and dystrophic). They are differentiated by the level of blister cleavage andsubdivided according to the pattern of genetic inheritance, morphology/topography oflesions and genetic mutation involved. After the Third International Consensus onDiagnosis and Classification of Inherited EB, there was the addition of a fourth entityto the group of inherited EB - Kindler syndrome (KS) previously considered aphotosensitive poikiloderma.3,4 Currently, over 30 phenotypiccally andgenetically distinct nosological entities have been described (Table 1).1,3Figure 1 illustrates the key molecules involved inthe pathogenesis of EB.

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