Pyoderma gangrenosum
Pyoderma gangrenosum er en sjælden, inflammatorisk hudsygdom, hvor smertefulde pustler eller knuder udvikler sig til sår, der gradvist vokser. Sygdommen er ikke infektiøs. Behandlingen kan omfatte kortikosteroider, ciclosporin eller forskellige monoklonale antistoffer. Selvom den kan forekomme i alle aldersgrupper, rammer den oftest personer i 40‑ og 50‑årene.
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ReferencesPyoderma gangrenosum er en sjælden hudsygdom, der forårsager smertefulde sår med røde eller lilla kanter. Den klassificeres som en inflammatorisk lidelse og indgår i en gruppe kaldet neutrofile dermatoser. Årsagen til pyoderma gangrenosum er kompleks og involverer både medfødt og adaptiv immunitet hos genetisk disponerede personer. For nylig har forskere fokuseret på hårsækken som et potentielt udgangspunkt for sygdommen.
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 er en sjælden hudlidelse, der forårsager ekstremt smertefulde sår. Selvom vi endnu ikke forstår den præcise årsag, ved vi, at sygdommen involverer øget aktivitet af visse immunceller. Behandlingen er stadig udfordrende. Vi har flere lægemidler, der enten undertrykker immunsystemet eller modificerer dets aktivitet. Samtidig fokuserer vi på sårbehandling og smertehåndtering. Kortikosteroider og cyclosporin er ofte førstevalg, men på det seneste er der forsket mere i brugen af biologiske terapier, såsom TNF‑α‑hæmmere. Disse biologiske lægemidler foretrækkes i stigende grad, især hos patienter med andre inflammatoriske tilstande, og de anvendes tidligere i sygdomsforløbet.
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.
