Limits...
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.

Show MeSH

Related in: MedlinePlus

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

License
getmorefigures.php?uid=PMC3750879&req=5

f02: Appearance of lesions in patients with junctional epidermolysis bullosa

Mentions: Involvement of the oral mucosa, alopecia and anonychia are frequent.14-16Figure 2 shows lesions observed in patientswith JEB. Cases of JEB with congenital pyloric atresia and, more rarely, of otherportions of the gastrointestinal tract, have been described in the literature. Thisdisorder is associated with a significant risk of congenital abnormalities of thegenitourinary tract and infant/neonatal death. Although patients present with aphenotype virtually identical to that of EBS with pyloric atresia, intra-laminalucida cleavage of blisters characterizes these patients as having JEB.10,13,17


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 junctional epidermolysis bullosa
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f02: Appearance of lesions in patients with junctional epidermolysis bullosa
Mentions: Involvement of the oral mucosa, alopecia and anonychia are frequent.14-16Figure 2 shows lesions observed in patientswith JEB. Cases of JEB with congenital pyloric atresia and, more rarely, of otherportions of the gastrointestinal tract, have been described in the literature. Thisdisorder is associated with a significant risk of congenital abnormalities of thegenitourinary tract and infant/neonatal death. Although patients present with aphenotype virtually identical to that of EBS with pyloric atresia, intra-laminalucida cleavage of blisters characterizes these patients as having JEB.10,13,17

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