Teleangectasia emorragica ereditaria (Rendu-Osler)
Table of Contents
Che cos’è la teleangectasia emorragica ereditaria?
La teleangectasia emorragica è una malformazione vascolare ereditaria, a trasmissione autosomica dominante, che colpisce soprattutto il sistema venoso le cui pareti si indeboliscono e si dilatano. La teleangectasia emorragica ereditaria è causata da diverse mutazioni in uno dei due geni finora identificati e localizzati l’uno sul cromosoma 9 e l’altro sul cromosoma 12.
Come si trasmette?
La teleangectasia emorragica ereditariaviene ereditata in maniera autosomica dominantecon una frequenza di circa 1:50.00ed è caratterizzata da lesioni cutanee e mucose di tipo puntiforme o nodulariche diventano sanguinanti quando traumatizzate. Le lesioni si trovano soprattutto nellalingua, nella regione periorale, sotto la lingua, sulla mucosa nasale, sulle labbra.
Quali sono i sintomi della teleangectasia emorragica ereditaria?
Questa malformazione vascolare colpisce entrambi i sessi. Durante l’infanzia compaionoteleangectasie sul volto(labbra, guancia, mucose nasali, lingua, orofaringe). Le teleangiectasie si presentano conpiccole chiazze rossastre di qualche mm di diametro, leggermente rilevate che scompaiono alla vitropressione. Nel 5-10% dei casi esiste una fistola arterovenosa del polmone.
Complicazioni
Le emorragie non compaiono in genere prima dei 30-40 anni. Si osservano epistassi, emorragie digestive micro o macroscopiche che conducono talvolta all’anemia ferropriva.
Diagnosi
La diagnosi è fondata sull’esistenza di teleangectasie sul volto, di complicazioni emorragiche a partire da 30-40 anni e da un anamnesi familiare.
Terapia
Cauterizzazione della mucosa nasale in caso di epistassi. Le epistassi richiedono un trattamento medico aggressivo e possibilmente una risoluzione chirurgica. Tamponamenti nasali, agenti emostatici locali, derivati ormonali (estrogeni) impiegati in sede e, nei casi più gravi, la plastica del setto nasale sono i più utili mezzi terapeutici impiegati in questo tipo di pazienti.
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