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J Dtsch Dermatol Ges
; 19(7): 983-986, 2021 07.
Artigo
em Inglês
| MEDLINE
| ID: mdl-33951274
RESUMO
If blistering occurs in childhood, the possibility of hereditary epidermolysis bullosa should be considered even if the symptoms are mild. Besides clinical and histological examination, molecular genetic screening is diagnostically relevant. For localized forms, symptomatic, topical therapy options are currently still the primary choice. Of particular interest is the new option of topical therapy with diacerein 1 % cream. In the case of a pronounced clinical picture with extracutaneous organ involvement, multidisciplinary management is required. In the future, new forms of therapy such as autologous epidermal stem cell transplantation and gene therapeutic procedures may be applied. Human genetic counselling is indispensable.
Assuntos
Epidermólise Bolhosa Distrófica , Epidermólise Bolhosa , Vesícula , Epidermólise Bolhosa/diagnóstico , Epidermólise Bolhosa/genética , Epidermólise Bolhosa/terapia , Epidermólise Bolhosa Distrófica/diagnóstico , Epidermólise Bolhosa Distrófica/genética , Epidermólise Bolhosa Distrófica/terapia , Terapia Genética , Humanos , Doenças Raras/diagnóstico , Doenças Raras/genética , Doenças Raras/terapia
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