Management of pyoderma gangrenosum

Abstract

Pyoderma gangrenosum (PG) is an uncommon ulcerative skin disease often associated with underlying systemic diseases. Its pathogenesis is unknown, although immune pathways have been implicated. Targeted therapy is therefore lacking and currently treatment is largely empirical and consists of corticosteroids and ciclosporin first line. This paper reviews the current and emerging knowledge about PG. PG occurs with an incidence of 3–10 per million per year. The ulcers are exquisitely painful and characteristically have a necrotic centre with irregular overhanging bluish borders. Around half of cases are associated with underlying systemic disease, most commonly inflammatory bowel disease, rheumatoid arthritis and haematological malignancies; the remaining cases are idiopathic. The pathogenesis is unknown, but the most widely supported theory is immunological, and biopsies of lesions show a predominantly neutrophilic infiltrate. Several aberrant immune processes have been described, with neutrophils and their recruitment to sites of inflammation by cytokines taking an apparently important role. Topical and systemic therapies are both vital aspects of treatment, and in recent years, immune modulators have been used with increasing success, with an emerging role for anti-tumour necrosis factor alpha agents such as the monoclonal antibody infliximab. Although uncommon, PG causes significant morbidity to those it affects. Further research is needed into the disease pathogenesis, and adequate targeted treatment.

Gene Therapy for Pyoderma Gangrenosum: Optimal Transfection Conditions and Effect of Drugs on Gene Delivery in the HaCaT Cell Line Using Cationic Liposomes

“…This study is the first to investigate the effect on gene transfection of three drugs which are commonly used to treat PG, and has shown that a significantly positive effect was exerted by hydrocortisone. Hydrocortisone is a glucocorticoid, one of the most powerful immunosuppressive and anti-inflammatory groups of drugs [42], and is a first-line therapy for PG [2,5,43]. The effect of hydrocortisone on keratinocyte survival and proliferation is disputed.…”

“…The pathogenesis is not known, but inflammatory and immunological processes are thought to be the underlying mechanisms [3]. Current treatment is mostly empirical, with corticosteroids and other immunosuppressants as first line [4,5]. Several therapies may need to be trialled to achieve remission of the disease, all of which have potentially severe toxicities.…”

Pyoderma Gangrenosum and Surgery: Case Report

“…1 Hastalığın insidansı tam olarak bilinmemekle birlikte yılda 3-10/1.000.000 olarak belirtilmektedir. Bazı kaynaklarda hastalığın her iki cinsiyeti eşit olarak etkilediği bildirilirken, hastalığın kadınlarda daha sık görüldüğünü bildiren kaynaklar da bulunmaktadır.…”

“…Bazı kaynaklarda hastalığın her iki cinsiyeti eşit olarak etkilediği bildirilirken, hastalığın kadınlarda daha sık görüldüğünü bildiren kaynaklar da bulunmaktadır. 1 PG klinik olarak ülseratif, püstüler, büllöz, vejetatif ve peristomal olmak üzere beş alt gruba ayrılmaktadır, ancak daha nadir görülen varyantlar da tanımlanmıştır. 2 Klasik tipinde bir ya da birkaç küçük püstül ile başlayıp hızla büyüyen iyi sınırlı, kenarı mavi-mor renkli derin ve ağrılı Piyoderma Gangrenozum ve Cerrahi Ö ÖZ ZE ET T Piyoderma gangrenozum (PG) etiyolojisi bilinmeyen, nötrofilik infiltrasyonla karakterize, nadir görülen inflamatuar bir hastalıktır.…”

“…Ülser merkezinde dermis ve subkütan doku boyunca inflamasyon, yoğun nötrofilik infiltrasyonla birlikte histiyosit ve makrofaj birikimi gö-rülmektedir. 1 PG hastalarının yaklaşık yarısında Crohn hastalığı, ülseratif kolit gibi inflamatuar barsak hastalıkları, romatoid artrit, lösemiler, kronik hepatit, psöriatik artrit, miyelofibrozis, lenfomalar ve sistemik lupus eritematozus gibi sistemik hastalıklarla birliktelik tanımlanmıştır.…”