Abstract:2]. Available from:. https://www.fda.gov/ regulatory-information/search-fda-guidance-documents/intravascular-catheters-wires-and-delivery-systems-lubricious-coatingslabeling-considerations. 6. Falanga V, Fine MJ, Kapoor WN. The cutaneous manifestations of cholesterol crystal embolization.
Pyoderma gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers. We aimed to evaluate retrospectively the demographic, clinical characteristics, treatment data and factors affecting treatment responses of patients followed up with diagnosis of PG in 12 tertiary care centers from 8 different provinces in Turkey between the years 2012–2022. We included a total of 239 patients of whom 143 (59.8%) were female and 96 (40.2%) were male, with an average age of 54.2 ± 17.4 years. At least one comorbidity associated with PG was found in 51.5% of our patients. The most common comorbidities were inflammatory arthritis (18.8%) and inflammatory bowel disease (IBD) (15.9%). The most common treatment was systemic steroids (75.7%). Among these patients 50.8% used systemic steroids as the sole systemic agent, while 49.2% used at least one adjuvant immunosuppressive agent. The general characteristics of our patients who responded well to systemic steroid treatment were those with a late onset of disease, shorter disease duration, fewer lesions, single region involvement, negative wound culture results, negative pathergy, and those without inflammatory arthritis and IBD (all p < 0.05). Biological agents were used in 18.4% of our patients in our study. The general characteristics of the patients who switched to biologics as a second or third line treatment are those with early disease onset, younger patients, males, those with autoinflammatory diseases, those with IBD and hidradenitis suppurativa and pathergy positive ones (all p < 0.05). Our study had compelling data concerning two specific patient groups: Pathergy positivite and early onset (onset age < 30) PG groups. We think that investigation for accompanying autoinflammatory diseases at the time of diagnosis in these two groups is necessary and the patients in these two groups are more resistant to treatment, necessitating more complicated treatments. We believe that the results of our multicenter study will contribute to the literature.
Pyoderma gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers. We aimed to evaluate retrospectively the demographic, clinical characteristics, treatment data and factors affecting treatment responses of patients followed up with diagnosis of PG in 12 tertiary care centers from 8 different provinces in Turkey between the years 2012–2022. We included a total of 239 patients of whom 143 (59.8%) were female and 96 (40.2%) were male, with an average age of 54.2 ± 17.4 years. At least one comorbidity associated with PG was found in 51.5% of our patients. The most common comorbidities were inflammatory arthritis (18.8%) and inflammatory bowel disease (IBD) (15.9%). The most common treatment was systemic steroids (75.7%). Among these patients 50.8% used systemic steroids as the sole systemic agent, while 49.2% used at least one adjuvant immunosuppressive agent. The general characteristics of our patients who responded well to systemic steroid treatment were those with a late onset of disease, shorter disease duration, fewer lesions, single region involvement, negative wound culture results, negative pathergy, and those without inflammatory arthritis and IBD (all p < 0.05). Biological agents were used in 18.4% of our patients in our study. The general characteristics of the patients who switched to biologics as a second or third line treatment are those with early disease onset, younger patients, males, those with autoinflammatory diseases, those with IBD and hidradenitis suppurativa and pathergy positive ones (all p < 0.05). Our study had compelling data concerning two specific patient groups: Pathergy positivite and early onset (onset age < 30) PG groups. We think that investigation for accompanying autoinflammatory diseases at the time of diagnosis in these two groups is necessary and the patients in these two groups are more resistant to treatment, necessitating more complicated treatments. We believe that the results of our multicenter study will contribute to the literature.
“…In a large single-centre study, mycophenolate sodium and mofetil were used in a considerable proportion of patients (75/118) and were associated with a lower rate of adverse events than cyclosporine. The same authors combined MMF with low dose cyclosporine in some cases [ 63 ].…”
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 can be associated with haematological malignancies but rarely a myeloproliferative neoplasm. A review of requests for molecular detection of myeloproliferative neoplasm driver mutations in patients with pyoderma gangrenosum was performed and revealed that testing for these mutations is unwarranted in cases where there are no clinical, haematological or morphological features of a myeloproliferative neoplasm present.
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