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EPIDERMOLISI BOLLOSA EREDITARIA
Codice esenzione : RN0570

EPIDERMOLISI BOLLOSA LETALIS CON ATRESIA PILORICA fa riferimento a EPIDERMOLISI BOLLOSA EREDITARIA


Gruppo di malattie geneticamente determinate caratterizzate da vescicolazioni della cute e delle mucose. E. B. Semplice: e caratterizzata da un'ereditarieta autosomica dominante e dalla formazione di bolle severe che guariscono senza cicatrice.E. B. Giunzionale: esordisce alla nascita o durante il periodo neonatale ed ha una trasmissione autosomica recessiva. E' caratterizzata dalla formazione di vescicole generalizzate, denudazione estesa e separazione e clivaggio delle membrane delle cellule basali dalla membrane basale.E. B. Distrofica: e caratterizzata da atrofia delle aree con vescicole, ulcerazioni severe e cambiamenti delle unghie. E' piu spesso presente alla nascita o precocemente in infanzia e si presenta sia come tratto autosomico dominate che recessivo . - Medline Thesaurus

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