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Pyoderma gangrenosum (PG) is an extremely rare case of sterile necrotic ulcerative disease associated with malnutrition as a predisposition factor. It is unclear, though, whether dilated cardiomyopathy, which affects blood flow and results in stenosis in the arteries, could play a role as an etiology. In this study, a case of pyoderma gangrenosum in a 10-year-old boy complicated by dilated cardiomyopathy, a previous history of cerebrovascular disease, and a malnourished condition were reported. The patient was reported to have exudative necrotic lesions in both legs. Lesions began as small, multiple, itchy lesions on both legs, which later became blisters and scuffed, and progressed into painful, peeled-off lesions with pus, bleeding, redness around lesions, and maggots within a month. A high fever was an accompanying symptom. The multidisciplinary team was involved to provide a comprehensive treatment for this patient. Antibiotics and necrotomy debridement were performed several times. Anticoagulant treatment was indicated as the coagulation markers were increased and echocardiography suggested thrombus in the left ventricle. The underlying condition that increases the risk of pyoderma gangrenosum should be corrected. The patient was discharged after a clinical improvement, although the continuation of outpatient monitoring was required. Our report suggests that a chronic condition of dilated cardiomyopathy that affects normal blood flow leads to malnutrition, the formation of thrombus, and stenosis of a peripheral artery, all of which contributed to pyoderma gangrenosum. Therefore, early surgical treatment, antibiotic administration, and anticoagulant treatment were recommended.

BACKGROUND: Pyoderma Gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers.
OBJECTIVE: We aimed to evaluate the demographic, clinical characteristics, treatments and factors affecting the treatment responses of patients with PG.
METHODS: We performed a multicenter study of 12 tertiary care centers. We analyzed the data of the patients who were followed up with a diagnosis of PG between the years 2012‒2022 retrospectively.
RESULTS: We included a total of 239 patients of whom 143 were female and 96 were male, with an average age of 54.2 ± 17.4 years. The most common treatment was systemic steroids (n = 181, 75.7%). Among these patients, 50.8% (n = 92) used systemic steroids as the sole systemic agent, while 49.2% (n = 89) used at least one adjuvant immunosuppressive agent. The independent factors determined in regression analysis to influence response to systemic steroids positively were disease onset age ≥ 30-years, negative pathergy, absence of leukocytosis, negative wound culture, presence of a single lesion, and absence of upper extremity involvement. Biological agents were used in 18.4% (n = 44) of the patients in the present study. We also analyzed pathergy positive PG and early onset (onset age < 30) PG separately due to their distinct clinical features which were revealed during statistical analysis.
CONCLUSIONS: Retrospective nature of the present study.
CONCLUSIONS: Analyses of the factors influencing treatment responses are addressed in this study. Also, we concluded that investigation for accompanying autoinflammatory diseases of pathergy positive PG and early onset PG is necessary and the patients in these two groups are more resistant to treatment, necessitating more complicated treatments.

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BACKGROUND: Hidradenitis suppurativa (HS) is a debilitating cutaneous disease characterized by severe painful inflammatory nodules/abscesses. At present, data regarding the epidemiology and pathophysiology of this disease are limited.
OBJECTIVE: To define the prevalence and comorbidity associations of HS.
METHODS: This was a cross-sectional study of EPICTM Cosmos© examining over 180 million US patients. Prevalences were calculated by demographic and odds ratios (OR) and identified comorbidity correlations.
RESULTS: All examined metabolism-related, psychological, and autoimmune/autoinflammatory (AI) diseases correlated with HS. The strongest associations were with pyoderma gangrenosum [OR 26.56; confidence interval (CI): 24.98-28.23], Down syndrome (OR 11.31; CI 10.93-11.70), and polycystic ovarian syndrome (OR 11.24; CI 11.09-11.38). Novel AI associations were found between HS and lupus (OR 6.60; CI 6.26-6.94) and multiple sclerosis (MS; OR 2.38; CI 2.29-2.48). Cutaneous malignancies were largely not associated in the unsegmented cohort; however, among Black patients, novel associations with melanoma (OR 2.39; CI 1.86-3.08) and basal cell carcinoma (OR 2.69; CI 2.15-3.36) were identified.
CONCLUSIONS: International Classification of Diseases (ICD)-based disease identification relies on coding fidelity and diagnostic accuracy.
CONCLUSIONS: This is the first study to identify correlations between HS with melanoma and basal cell carcinoma (BCC) among Black patients as well as MS and lupus in all patients with HS.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare ulcerative skin condition with no current standardized outcomes or outcome measures. With a rich investigational therapeutic pipeline, standardization of outcomes and improvement of data quality and interpretability will promote the appropriate and consistent evaluation of potential new therapies. Core outcome sets (COS) are agreed, standardized sets of outcomes that represent the minimum that should be measured and reported in all clinical trials of a specific condition.
OBJECTIVE: To identify and reach a consensus on which domains (what to be measured) should be included in the Understanding Pyoderma Gangrenosum: Review and Analysis of Disease Effects (UPGRADE) core domain set for clinical trials in PG.
METHODS: Collaborative discussions between patients and PG experts, and a systematic review of the literature identified items and prospective domains. A three-round international eDelphi exercise was performed to prioritize the domains and refine the provisional items (consensus: ≥ 70% of participants rating a domain as \'extremely important\' and < 15% of participants voting \'not important\'), followed by an international meeting to reach consensus on the core domain set (consensus: < 30% disagreement). Item-generation discussions and consensus meetings were hosted via online videoconferences. The eDelphi exercise and consensus voting were performed using Qualtrics survey software. Participants were adults with PG, healthcare professionals, researchers and industry representatives.
RESULTS: Collaborative discussions and systematic reviews yielded 115 items, which were distilled into 15 prospective domains. The eDelphi exercise removed the three lowest-priority domains (\'laboratory tests\', \'treatment costs\' and \'disease impact on family\') and ranked \'pain\', \'quality of life\' and \'physical symptoms\' as the highest-priority prospective domains. Consensus was reached on the domains of \'pain\', \'quality of life\' and \'clinical signs\'. The domain of \'disease course/disease progression\' narrowly failed to reach consensus for inclusion in the core set (32% of participants voted \'no\'). Refinement of this domain definition will be required and presented for consideration at future consensus meetings.
CONCLUSIONS: The UPGRADE core domain set for clinical trials in PG has been agreed by international multistakeholder consensus. Future work will develop and/or select outcome measurement instruments for these domains to establish a COS.

Pyoderma gangrenosum (PG) is a rare inflammatory condition with an immense disease burden that remains understudied. With limited approved treatments and low-quality clinical evidence, PG continues to have poor patient outcomes. Unfortunately, improvement in PG treatments and patient care is based on additional research endeavors that can only be developed from existing high-quality data. The following protocol outlines the development of the Minimum Data Set for Treatment Effectiveness in Pyoderma gangrenosum (MIDSTEP), a core set of domains and domain items for the Pyoderma Gangrenosum Treatment Effectiveness (PyGaTE) international registry. The outcomes and benefits are focused on providing real-world data for physicians to improve their clinical decisions on PG treatment and inform clinical trial design, promoting clinical research among the international scientific community. MIDSTEP is a multi-phase project. The first phase will produce a domain item list from a literature review to take into the second phase which would finalize the core data set by an e-Delphi exercise. There will be a single stakeholder group participating together in the e-Delphi consisting of PG experts (healthcare providers, researchers, methodologists, industry representatives, and regulators), ulcerative PG patients, and PG patient advocates. The methodology outlined in the protocol is a systematic method based on several guidelines through COMET and established dermatologic registries and outcome sets with systematic methodologies of their own. The third phase will identify the instruments for the items, the \'when to measure\' the items, and the platform for the registry. The last phase is the implementation and continued maintenance of the international registry PyGaTE. By solidifying a consensus on standardized outcomes and collecting information on PG treatment effectiveness in a centralized database, existing treatments can be compared more systematically and analyzed with increased evidence. MIDSTEP and the PyGaTE international registry will have the ambitious goal to generate and disseminate real-world data that can be used by all stakeholders to improve health outcomes for PG patients. Future potential for the outcome of this project includes the development of a gold-standard PG treatment.

Since pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis, epidemiological and clinical data on the disease are scarce. In this single-center retrospective study, we aim to evaluate the clinical characteristics, underlying systemic associations and treatment modalities in patients with PG in a university hospital between 2014 and 2022. It is known that PG most commonly affects the lower extremities, but extracutaneous involvement should also be kept in mind. PG is usually associated with various comorbidities that share a similar inflammatory pathogenesis with the disease. The prevalence of PG-related comorbidities varies in different studies, arthritis and solid organ malignancies were observed most frequently in the current study. Non-PG-related comorbidities including diabetes mellitus, hypertension and peripheral vascular disease can adversely affect wound healing and limit treatment options; therefore, a holistic approach to patients with PG is crucial. Consistent with literature, the mainstay of treatment for PG is systemic corticosteroids and cyclosporine. However, the implementation of biologic agents in treatment-resistant patients is an increasingly important issue in the literature. Antitumor necrosis factors (anti-TNFs) are the most commonly preferred biological therapies, and these agents seem to have paved the way for a paradigm shift in the treatment of PG. In the present study, a relatively high per cent of (23.3%) patients treated with anti-TNFs, most commonly infliximab (87.5%). Recurrence was observed in 46.7% of our patients in the follow-up period and the relapse rate was found to be higher in patients using multiple systemic agents compared to those using single agents (64.7% vs 23.1%, P < .05). In conclusion, we emphasize that early diagnosis and treatment by considering the patient\'s comorbidities are important in preventing complications, and biologic treatments seem particularly promising in treatment-resistant patients.

UNASSIGNED: Pyoderma gangrenosum (PG) is a rare, neutrophilic dermatosis that is a well-established extraintestinal manifestation (EIM) of inflammatory bowel disease. The clinical implications of developing PG in patients with ulcerative colitis (UC) who undergo total proctocolectomy colectomy and ileal pouch anal anastomosis (TPC-IPAA) surgery remain unknown.
UNASSIGNED: Study participants were selected from patients enrolled in the Carlino Family Inflammatory Bowel and Colorectal Disease Biobank between 1998 and 2021 with a pre-colectomy diagnosis of UC and who underwent TPC-IPAA surgery. A retrospective study comparing patients with PG and those without PG was performed. The outcomes measured included the development of pouchitis, pouchitis classification, presence of pouch fistula, anal fistula, anal stenosis, and pouch failure.
UNASSIGNED: In this study, 357 IPAA patients were included, 10 of whom suffered PG. Patients with PG and without PG had similar demographics and clinical characteristics. Both groups had similar rates of pouchitis (80% in PG patients and 64% in patients without PG, P = .504). However, IPAA patients with PG had a higher risk of developing pouch fistula (50% vs 10%, P = .002), anal fistula (40% vs 12%, P = .031), and Crohn\'s-like disease of the pouch (70% vs 15%, P = .003) compared to patients without PG. Patients who developed PG prior to their first episode of pouchitis were more likely to eventually experience pouch failure (odds ratio: 20.7, 95% confidence interval: 3.9, 110.7, q = 0.003 after false discovery rate adjustment).
UNASSIGNED: Among UC patients who undergo TPC-IPAA surgery, the development of PG portends poor pouch outcomes and is predictive of pouch failure.

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