At present, there are no standardized guidelines for determining patient eligibility for pyoderma gangrenosum (PG) clinical trials. Thus, we aim to determine which clinical features, histopathological features, or laboratory features should be included in active ulcerative PG clinical trial eligibility criteria for treatment-naïve patients and patients already treated with immunomodulating medications (treatment-exposed patients). This study employed 4 rounds of the Delphi technique. Electronic surveys were administered to 21 international board-certified dermatologists and plastic surgeon PG experts (June 2022-December 2022). Our results demonstrated that for a patient to be eligible for a PG trial, they must meet the following criteria: (i) presence of ulcer(s) with erythematous/violaceous undermining wound borders, (ii) presence of a painful or tender ulcer, (iii) history/presence of rapidly progressing disease, (iv) exclusion of infection and other causes of cutaneous ulceration, (v) biopsy for H&E staining, and (vi) a presence/history of pathergy. These criteria vary in importance for treatment-naïve versus treatment-exposed patients. Given the international cohort, we were unable to facilitate live discussions between rounds. This Delphi consensus study provides a set of specific, standardized eligibility criteria for PG clinical trials, thus addressing one of the main issues hampering progress toward Food and Drug Administration approval of medications for PG.

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.

暂无摘要。

We found that the systematic use of Delphi consensus diagnostic criteria resulted in substantial changes in management patterns in cases of suspected pyoderma gangrenosum (PG), including a reduction in systemic corticosteroid use, and significantly improved clinical outcomes. This improvement may have resulted from a combination of reduced misdiagnosis, optimized management and reduced iatrogenic harm. Increased utilization of validated diagnostic criteria for PG could optimize provider heuristics, increase diagnostic accuracy, and optimize management and clinical outcomes.

Extra-intestinal manifestations (EIMs) are a common complication of inflammatory bowel diseases (IBD), affecting up to half of the patients. Despite their high prevalence, information on standardised definitions, diagnostic strategies, and treatment targets is limited.
As a starting point for a national EIM study network, an interdisciplinary expert panel of 12 gastroenterologists, 4 rheumatologists, 3 ophthalmologists, 6 dermatologists, and 4 patient representatives was assembled. Modified Delphi consensus methodology was used. Fifty-four candidate items were derived from the literature review and expert opinion focusing on five major EIMs (erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis) were rated in three voting rounds.
For use in a clinical practice setting and as part of the creation of a prospective registry of patients with EIMs, the panel developed definitions for erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis; identified the appropriate and optimal subspecialists to diagnose and manage each; provided methods to monitor disease course; offered guidance regarding monitoring intervals; and defined resolution and recurrence.
Consensus criteria for appropriate and optimal means of diagnosing and monitoring five EIMs have been developed as a starting point to inform clinical practice and future trial design. Key findings include straightforward diagnostic criteria, guidance regarding who can appropriately and optimally diagnose each, and monitoring options that include patient and physician-reported outcomes. These findings will be used in a national multicenter study network to optimise the management of EIMs.

Pyoderma gangrenosum following free tissue transfer for breast reconstruction is rare. This unusual ulcerative condition is frequently misdiagnosed, leading to inappropriate debridement and escalation of the subsequent wound through pathergy. Once diagnosed, treatment with immunosuppressive agents, including corticosteroids, results in an initial rapid response, but prolonged treatment is required. There is a paucity of literature regarding how to approach future surgery.
This was a retrospective case review from a single center over a 17-year period. All patients diagnosed with postsurgical pyoderma gangrenosum after free tissue transfer from the abdomen for breast reconstruction were included.
Of 456 free tissue transfers from the abdomen for breast reconstruction, 8 women who underwent 13 free flaps were diagnosed with postsurgical pyoderma gangrenosum in 10 flaps. The surgeries performed included transverse rectus abdominis muscle (n = 5), deep inferior epigastric perforator (n = 4) and superficial inferior epigastric artery (n = 4) flaps. Mean age at diagnosis was 52.8 years, and 3 patients had preexisting autoimmune conditions: type 2 diabetes mellitus, dermatomyositis, and Graves disease. The mean time of presentation of wound symptoms was 3.9 days after surgery, and mean time diagnosis was made was 9.4 days.
Pyoderma gangrenosum after autologous breast reconstruction is a rare, but serious, complication that is worsened by misdiagnosis and inappropriate debridement. We present a case series of 8 patients and emphasize the importance of early recognition and treatment with immune suppression. We include a treatment algorithm to manage these patients, once the diagnosis is suspected. Future surgery can be considered with a fully informed patient and careful collaboration with dermatology colleagues.

Pyoderma gangrenosum is a rare inflammatory skin condition that is difficult to diagnose. Currently, it is a \"diagnosis of exclusion,\" a definition not compatible with clinical decision making or inclusion for clinical trials.
To propose and validate diagnostic criteria for ulcerative pyoderma gangrenosum.
Diagnostic criteria were created following a Delphi consensus exercise using the RAND/UCLA Appropriateness Method. The criteria were validated against peer-reviewed established cases of pyoderma gangrenosum and mimickers using k-fold cross-validation with methods of multiple imputation.
Delphi exercise yielded 1 major criterion-biopsy of ulcer edge demonstrating neutrophilic infiltrate-and 8 minor criteria: (1) exclusion of infection; (2) pathergy; (3) history of inflammatory bowel disease or inflammatory arthritis; (4) history of papule, pustule, or vesicle ulcerating within 4 days of appearing; (5) peripheral erythema, undermining border, and tenderness at ulceration site; (6) multiple ulcerations, at least 1 on an anterior lower leg; (7) cribriform or \"wrinkled paper\" scar(s) at healed ulcer sites; and (8) decreased ulcer size within 1 month of initiating immunosuppressive medication(s). Receiver operating characteristic analysis revealed that 4 of 8 minor criteria maximized discrimination, yielding sensitivity and specificity of 86% and 90%, respectively.
This Delphi exercise produced 1 major criterion and 8 minor criteria for the diagnosis of ulcerative pyoderma gangrenosum. The criteria may serve as a guideline for clinicians, allowing for fewer misdiagnoses and improved patient selection for clinical trials.

Pyoderma gangrenosum (PG) is an atypical ulceration of the skin with unknown aetiology, usually associated with autoimmune systemic illnesses and haematological malignancies. Diagnosis is based on clinical suspicion and exclusion of other conditions. Treatment options vary greatly, ranging from conservative local and systemic immunosuppression to surgical measures, including amputation, but none is shown to be universally effective. Currently no guideline regarding escalation of treatment exists. Based on a review of the current literature and three illustrative cases of PG, a working treatment guideline is presented for wound practitioners.