Pyoderma gangrenosum
https://en.wikipedia.org/wiki/Pyoderma_gangrenosum
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References
Pyoderma Gangrenosum: An Updated Literature Review on Established and Emerging Pharmacological Treatments 35606650 NIH
Pyoderma gangrenosum azaleko gaixotasun arraroa da, ertz gorri edo moreekin ultzera mingarriak eragiten dituena. Hanturazko gaixotasun gisa sailkatzen da eta dermatosi neutrofiliko izeneko talde baten parte da. Pyoderma gangrenosum -ren kausa konplexua da, genetikoki joera duten pertsonengan berezko eta moldapenezko immunitatearekin arazoak dakartza. Berriki, ikertzaileek ile-folikuluan arreta jarri dute gaixotasunaren abiapuntu potentzial gisa.
Pyoderma gangrenosum is a rare inflammatory skin disease classified within the group of neutrophilic dermatoses and clinically characterized by painful, rapidly evolving cutaneous ulcers with undermined, irregular, erythematous-violaceous edges. Pyoderma gangrenosum pathogenesis is complex and involves a profound dysregulation of components of both innate and adaptive immunity in genetically predisposed individuals, with the follicular unit increasingly recognized as the putative initial target.
Pyoderma Gangrenosum: Treatment Options 37610614 NIH
Pyoderma gangrenosum azaleko gaixotasun arraroa da, ultzera oso mingarriak eragiten dituena. Bere kausa guztiz ulertzen ez dugun arren, badakigu zelula immune batzuen jarduera areagotzen duela. Gaixotasuna tratatzea ez da oraindik erraza. Sistema immunologikoa kentzen edo haren jarduera aldatzen duten hainbat sendagai ditugu. Horiekin batera, zauriak tratatzen eta mina kudeatzen ere jartzen dugu arreta. Kortikoideak eta ziklosporina izan ohi dira tratamendurako lehen aukera, baina azkenaldian, TNF-α inhibitzaileak bezalako terapia biologikoak erabiltzeari buruzko ikerketa gehiago egin da. Biologiko hauek gero eta hobetsiagoak dira, batez ere hanturazko beste baldintza batzuk dituzten pazienteetan, eta gaixotasunaren prozesuan lehenago erabiltzen ari dira.
Pyoderma gangrenosum is a rare neutrophilic dermatosis that leads to exceedingly painful ulcerations of the skin. Although the exact pathogenesis is not yet fully understood, various auto-inflammatory phenomena with increased neutrophil granulocyte activity have been demonstrated. Despite the limited understanding of the pathogenesis, it is no longer a diagnosis of exclusion, as it can now be made on the basis of validated scoring systems. However, therapy remains a major multidisciplinary challenge. Various immunosuppressive and immunomodulatory therapies are available for the treatment of affected patients. In addition, concomitant topical pharmacologic therapy, wound management and pain control should always be addressed. Corticosteroids and/or cyclosporine remain the systemic therapeutics of choice for most patients. However, in recent years, there has been an increasing number of studies on the positive effects of biologic therapies such as inhibitors of tumour necrosis factor-α; interleukin-1, interleukin-17, interleukin-23 or complement factor C5a. Biologics have now become the drug of choice in certain scenarios, particularly in patients with underlying inflammatory comorbidities, and are increasingly used at an early stage in the disease rather than in therapy refractory patients.