Pyoderma gangrenosum
Pyoderma gangrenosum je zriedkavé zápalové ochorenie kože, pri ktorom sa bolestivé pustuly alebo uzliny stávajú vredmi, ktoré sa postupne zväčšujú. Pyoderma gangrenosum nie je infekčné. Liečba môže zahŕňať kortikosteroidy, cyklosporín alebo rôzne monoklonálne protilátky. Hoci môže postihnúť ľudí v akomkoľvek veku, väčšinou postihuje ľudí vo veku 40 až 50 rokov.
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Na nohe osoby s ulceróznou kolitídou.
References
Pyoderma gangrenosum je zriedkavé ochorenie kože, ktoré spôsobuje bolestivé vredy s červenými alebo purpurovými okrajmi. Je klasifikovaný ako zápalové ochorenie a je súčasťou skupiny nazývanej neutrofilné dermatózy. Príčina pyoderma gangrenosum je komplexná a zahŕňa problémy s vrodenou aj adaptívnou imunitou u ľudí, ktorí sú geneticky náchylní. Nedávno sa vedci zamerali na vlasový folikul ako potenciálny východiskový bod ochorenia.
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 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 je zriedkavé kožné ochorenie spôsobujúce extrémne bolestivé vredy. Aj keď úplne nerozumieme jej príčine, vieme, že zahŕňa zvýšenú aktivitu určitých imunitných buniek. Liečba choroby stále nie je jednoduchá. Máme rôzne lieky, ktoré potláčajú imunitný systém alebo upravujú jeho činnosť. Okrem toho sa zameriavame aj na ošetrovanie rán a zvládanie bolesti. Kortikosteroidy a cyklosporín sú často prvou voľbou liečby, ale v poslednej dobe sa viac skúmalo používanie biologických terapií, ako sú inhibítory TNF-α. Tieto biologické lieky sú čoraz viac preferované, najmä u pacientov s inými zápalovými stavmi, a používajú sa skôr v procese ochorenia.
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.
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.
