La lebbra è una malattia infettiva cronica che si manifesta dopo il contagio con Mycobacterium leprae in individui predisposti. Il periodo di incubazione della lebbra va da 2 a 5 anni per le forme tubercoloidi e 8-12 per le forme lepromatose. Nelle manifestazioni cliniche sono coinvolti soprattutto la cute, i nervi periferici, le mucose delle vie alte respiratorie e gli occhi. Il contagio può avvenire per trasmissione diretta mediante contatto tra mucose orali e rapporti sessuali, oppure per via indiretta mediante goccioline di saliva o secrezioni di varia natura.
Epidemiologia
A livello globale il numero dei casi di lebbraè in declino. La lebbra è presente in molti paesi dell’Asia, dell’America Latina e dell’Africa. In pratica si stima che nel mondo vi siano ancora 1-2 milioni di malati. In Europa i rari casi sono di importazione e bisogna tenere presente questo dato in virtù di un’evoluzione verso una società multietnica. Nel 2009 sono stati riportati circa 200 nuovi casi negli Stati Uniti. Aree ad alta endemicità sono ancora presenti in Angola, Brasile, Repubblica Centrafricana, Repubblica Democratica del Congo, India, Madagascar, Mozambico, Nepal, Repubblica unità di Tanzania.
Come avviene il contagio?
L’uomo è il principale riservoir della lebbrae la fonte di contagio. Sono contagiosi soprattutto i soggetti affetti da lebbra lepromatosa i quali presentano un’elevata carica batterica a livello cutaneo e mucoso. Il microorganismo penetra attraverso la cute o le membrane mucose delle alte vie aeree; la via aerea è, probabilmente, più frequente, dal momento che i pazienti con la lebbra multibacillare lepromatosa eliminano molto i bacilli con le secrezioni nasali; scarsamente contagiosa è, al contrario, la forma paucibacillare tubercoloide. Sovraffollamento e scarse condizioni igieniche sono anche alla base della trasmissione.
Fattori genetici dell’ospite controllano la suscettibilità e l’espressione della malattia. L’estensione e la gravità delle manifestazioni cliniche dipendono, in parte dalla risposta immune dell’ospite e possono variare da completa resistenza (nessuna malattia), resistenza incompleta( forme indeterminate, tubercoloide) basta resistenza (forma lepromatosa).
Classificazione
La lebbra può essere classificata per tipo in base al numero di aree cutanee colpite in:
lebbra paucibacillare:≤ 5 lesioni cutanee senza evidenza di micobatteri
lebbra multibacillare:≥ 6 lesioni cutanee, batteri rilevati su campioni di lesioni cutanee, o entrambi
La lebbra può essere classificata anche in base alla risposta cellulare e riscontri clinici:
indeterminata (I)
tubercoloide (TT)
borderline tubercoloide (BT)
borderline (BB)
borderline lepromatosa (BL)
lepromatosa (LL)
La suddivisione correla con il numero delle lesioni e la carica batterica nel derma e nella mucosa nasale.
Le prime tre (I, TT e BT) corrispondono alla forma paucibacillare, altre alla forma multibacillare.
Sintomatologia
Nella maggioranza dei casi, i primi sintomi della malattia si manifestano a livello cutaneo; le lesioni cutanee consistono in:
macule
papule
placche
noduli e infiltrazioni diffuse
Nella forma tubercolare la sintomatologia è caratterizzata da alterazioni neurologiche e sono più colpiti i nervi più superficiali come:
ulnare
facciale
tibiale
Nella forma lepromatosa sono tipiche le lesioni cutanee nodulari:
eritematose
ipopigmentate
senza alterazioni cromatiche
più frequentemente localizzate agli arti e al volto (facies leonina) e l’interessamento della mucosa nasale con perforazione del setto nei casi avanzati. Frequente è il coinvolgimento dei testicoli che porta alla sterilità e quello oculare con conseguenza cecità. Gli esiti a lungo termine sono rappresentati da ulcere trofiche che facilitano le infezioni della cute e delle strutture ossee, dalle mutilazioni deturpanti e invalidanti.
La diagnosi della lebbraè difficile per la rarità della malattia e la scarsa familiarità con i sintomi. La diagnosi è clinica e istologica, può destare sospetto la presenza di una o più lesioni cutanee spesso con perdita della sensibilità, nervi ingrossati, deformità dovute a debolezza muscolare, bacilloscopia positiva per il bacillo di Hansen.
Terapia
Per la cura della lebbra si usano combinazioni di antibioticie poiché i batteri sono difficili da eliminare la terapia antibiotica dura a lungo, da un periodo di 6 mesi a molti anni in base alla gravità dell’infezione. Eventuali danni e deformità non regrediscono con la terapia e per tale motivo la diagnosi precoce risulta fondamentale.
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