Monoclonal gammopathy in the setting of Pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer.

Autoimmune, Autoinflammatory Disease and Cutaneous Malignancy Associations with Hidradenitis Suppurativa: A Cross-Sectional Study.

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. LIMITATIONS: International Classification of Diseases (ICD)-based disease identification relies on coding fidelity and diagnostic accuracy. CONCLUSION: 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.

Prevalence of Extraintestinal Manifestations in Inflammatory Bowel Disease: A Systematic Review and Meta-analysis.

BACKGROUND: Inflammatory bowel disease (IBD) is a multisystem disease impacting various body systems including musculoskeletal, ocular, skin, hepatobiliary, pulmonary, cardiac, and haematological systems. The extraintestinal manifestations of IBD are frequent, common in both ulcerative colitis (UC) and Crohn's disease (CD), and impact the morbidity and mortality of patients. METHODS: The Embase, Embase classic, and PubMed databases were searched between January 1979 and December 2021. A random effects model was performed to find the pooled prevalence of joint, ocular, and skin extraintestinal manifestations of UC and CD. RESULTS: Fifty-two studies were included that reported on 352 454 patients. The prevalence of at least 1 joint, ocular, or skin extraintestinal manifestation in all IBD, UC, and CD was 24%, 27%, and 35% respectively. The prevalence between UC and CD were similar for pyoderma gangrenosum and axial joint manifestations. Ocular manifestations were found to be more common in CD than in UC. Peripheral joint manifestations and erythema nodosum were found to be more common in CD than UC. DISCUSSION: To our knowledge, this is the first meta-analysis that reports on the prevalence of at least 1 joint, ocular, or skin extraintestinal manifestation in IBD. Our results are largely consistent with figures and statements quoted in the literature. However, our findings are based on significantly larger cohort sizes. Thus, our results have the potential to better power studies and more accurately counsel patients.

The prevalence of joint, ocular, or skin extraintestinal manifestations in IBD, UC, and CD was 24%, 27%, and 35% respectively. Ocular manifestations were more common in CD. Peripheral joint manifestations and erythema nodosum were more common in CD.

Association of All-Cause and Cause-Specific Mortality Risks With Pyoderma Gangrenosum.

Importance: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. Few studies have evaluated the mortality outcomes of patients with PG. Objective: To investigate all-cause and cause-specific mortality in patients with PG. Design, Setting, and Participants: This retrospective population-based cohort study used data from the National Health Insurance Service database of Korea and the National Death Registry of Korea from patients with incident PG (≥3 documented visits with an International Statistical Classification of Diseases and Related Health Problems, Tenth Revision [ICD-10] code of L88) during January 2003 to December 2019. For comparison, a 1:20 cohort of age-, sex-, insurance type-, and income level-matched controls without any documented visit with an ICD-10 code of L88 during the entire observation was included. Exposures: Pyoderma gangrenosum. Main Outcomes and Measures: The participants were observed from the index date to their death, emigration, or the end of the observation period to investigate all-cause and cause-specific mortality during the 17-year study period. Results: In total, 3386 patients with PG (1450 women [42.8%]; mean [SD] age, 57.8 [16.4] years) and 67 720 controls (29 000 women [42.8%]; mean [SD] age, 57.8 [16.3] years) were analyzed. All-cause mortality risk was greater in patients with PG than in controls (adjusted hazard ratio [aHR], 2.122; 95% CI, 1.971-2.285) after adjustment for smoking, drinking, body mass index, and comorbidities. Patients experienced greater mortality of infectious disease (aHR, 3.855; 95% CI, 2.640-5.628), neoplasm (aHR, 1.618; 95% CI, 1.363-1.920), hematologic disease (aHR, 12.298; 95% CI, 3.904-38.734), endocrine disease (aHR, 6.322; 95% CI, 5.026-7.953), neurologic disease (aHR, 2.039; 95% CI, 1.337-3.109), cardiovascular disease (aHR, 1.979; 95% CI, 1.645-2.382), respiratory disease (aHR, 1.757; 95% CI, 1.365-2.263), gastrointestinal disease (aHR, 2.278; 95% CI, 1.522-3.408), connective tissue disease (aHR, 8.685; 95% CI, 4.963-15.199), and kidney/urogenital disease (aHR, 3.617; 95% CI, 2.488-5.259) than controls. Compared with idiopathic PG (aHR, 2.062; 95% CI, 1.897-2.241), PG that was associated with solid organ cancer (aHR, 2.313; 95% CI, 1.956-2.737) and hematologic cancer (aHR, 8.330; 95% CI, 5.473-12.679) showed greater mortality, whereas PG that was associated with inflammatory bowel diseases showed a slightly better prognosis (aHR, 1.742; 95% CI, 0.964-3.148). Conclusions and Relevance: The results of this cohort study suggest that patients with PG had a higher all-cause and cause-specific mortality risk than the general population.

Diagnostic work-up and treatment in patients with pyoderma gangrenosum: retrospective analysis of US insurance claims-based data.

Pyoderma gangrenosum (PG) is a rare, and often challenging to diagnose, inflammatory disorder with relatively high rates of morbidity and mortality. Central to the diagnosis of PG is histologic evaluation and exclusion of other entities. Large-scale studies investigating the proportion of patients receiving a thorough diagnostic work-up, as well as prevalence studies regarding comorbidities and systemic treatment in PG using claims-based data, are sparse. Our objective was to identify patients diagnosed with PG and describe the diagnostic work-up and prevalence of common comorbidities and therapies in this population using claims-based data in a retrospective cohort study. In order to better understand practices of diagnostic work-up, we captured rates of skin biopsy, tissue culture, and/or surgical debridement prior to initial diagnosis. We also identified the prevalence of PG-associated comorbidities and initial immunosuppressive therapy given for PG. Of the 565 patients diagnosed with PG, 9.4% underwent skin biopsy, 8% tissue culture, and 1.4% both skin biopsy AND tissue culture prior to diagnosis. Inflammatory bowel disease was the most prevalent comorbidity (16.3%). The most common treatment administered was systemic corticosteroids (17%). Although practice guidelines explicitly delineate histology and exclusion of infection as important diagnostic criteria, only a minority of patients in this study underwent skin biopsy and/or tissue culture prior to receiving a diagnosis of PG, suggesting that patients may receive a diagnosis of PG without having tissue evaluation. Such discordance between practice guidelines and "real-world" practice inevitably increases the risk for misdiagnosis of PG and misdirected treatment with immunosuppressants for presumptive PG in cases of PG mimickers. Moreover, comorbidities associated with PG may occur, or be identified in, a lower proportion of patients as compared with what is reported in the existing literature. Study limitations include a population restricted to < 65 years with commercial insurance and the reliance upon ICD diagnostic coding to capture the population.

Clinicopathological features and prognosis of pyoderma gangrenosum in Korea: A single centre, retrospective, observational study over 20 years.

BACKGROUND: Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and prognosis of pyoderma gangrenosum. AIMS: We aimed to document clinicopathologic and prognostic data of the patients with pyoderma gangrenosum. METHODS: In this retrospective observational study, we reviewed case records of patients diagnosed with pyoderma gangrenosum between 1999-2019. RESULTS: Fifty-three patients were identified by reviewing medical records for skin biopsy; of these, 37 were men and 16 were women. Mean age at onset was 43.3 ± 18.5 years. The most frequently affected area was the lower extremities (60.4%), followed by the head and neck (17.0%). The most common subtype was ulcerative (47.2%), followed by bullous (22.6%). 30 cases had underlying diseases and the most common were malignancy (24.5%), followed by inflammatory bowel diseases (18.9%). The proportion of cases with history of trauma were significantly higher in post-operative type (100%) as compared to the bullous type (8.3%). Histologic features of granulation tissue were frequently found in post-operative type (66.7%) and bullous type (58.3%). Granulomas were predominantly found in bullous type (58.3%). Age <60 years appeared to be significantly associated with multiple lesions. Partial-to-complete remission was observed in 40 cases (75.5%). Nine (17.0%) cases experienced recurrence with a median progression-free period of six months (interquartile range of 3.0-9.0 months). Cases with underlying hematologic disorders and the bullous subtype were significantly associated with early recurrence. LIMITATIONS: This study was a single-centre study with a retrospective design. CONCLUSION: Pyoderma gangrenosum appears to have ethnic differences. Underlying haematologic disorders and bullous subtype have a worse prognosis. However, the type of histopathology did not correlate with the clinical outcome of pyoderma gangrenosum.

Comorbidity screening in hidradenitis suppurativa: Evidence-based recommendations from the US and Canadian Hidradenitis Suppurativa Foundations.

BACKGROUND: Hidradenitis suppurativa (HS) is associated with comorbidities that contribute to poor health, impaired life quality, and mortality risk. OBJECTIVE: To provide evidence-based screening recommendations for comorbidities linked to HS. METHODS: Systematic reviews were performed to summarize evidence on the prevalence and incidence of 30 comorbidities in patients with HS relative to the general population. The screening recommendation for each comorbidity was informed by the consistency and quality of existing studies, disease prevalence, and magnitude of association, as well as benefits, harms, and feasibility of screening. The level of evidence and strength of corresponding screening recommendation were graded by using the Strength of Recommendation Taxonomy (SORT) criteria. RESULTS: Screening is recommended for the following comorbidities: acne, dissecting cellulitis of the scalp, pilonidal disease, pyoderma gangrenosum, depression, generalized anxiety disorder, suicide, smoking, substance use disorder, polycystic ovary syndrome, obesity, dyslipidemia, diabetes mellitus, metabolic syndrome, hypertension, cardiovascular disease, inflammatory bowel disease, spondyloarthritis, and sexual dysfunction. It is also recommended to screen patients with Down syndrome for HS. The decision to screen for specific comorbidities may vary with patient risk factors. The role of the dermatologist in screening varies according to comorbidity. LIMITATIONS: Screening recommendations represent one component of a comprehensive care strategy. CONCLUSIONS: Dermatologists should support screening efforts to identify comorbid conditions in HS.

The inpatient burden of pyoderma gangrenosum and associated comorbidities in children in the United States.

Pyoderma gangrenosum is an ulcerating inflammatory condition defined pathologically by an abundance of neutrophils in the absence of infection. Often, hospital admission is necessary for rapidly progressing PG for wound care and adequate pain control. However, few large-scaled controlled studies exist examining hospitalizations for PG in the pediatric populations and the associated comorbidities. We sought to determine the prevalence, length of stay (LOS), cost of care, and any risk factors for admission and associated comorbidities in children hospitalized for PG in the U.S. Data were analyzed from the 2002 to 2012 National Inpatient Sample, including a 20% representative sample of all hospitalizations in the United States. The prevalence of hospitalization between 2002 and 2012 ranged from 2 to 11 per million hospitalized children. Hospitalization for PG was associated with older age, female gender, black race/ethnicity, the third quartile for household income, having 2-5 chronic conditions, being admitted to a micropolitan or a non-metro/micropolitan hospital and to a teaching hospital. Hospitalization with vs. without a primary diagnosis of PG was associated with significantly prolonged LOS. The total inflation-adjusted cost of care for hospitalization with a primary diagnosis of PG was $2,202,576; $200,234 per year). The geometric-mean cost of hospitalization was significantly higher in children with vs. without a primary diagnosis of PG. Children hospitalized for PG were found to have higher odds of thyroid disease, inflammatory bowel disease, hematologic malignancy, and other autoimmune disorders. While children are rarely hospitalized for PG, they have prolonged hospitalization, and clinical interventions need to be developed to prevent hospitalization for PG. Further, concomitant workup for other systemic comorbidities is also warranted at the time of diagnosis and throughout disease course.

Is pyoderma gangrenosum associated with solid malignancies? Insights from a population-based cohort study.

BACKGROUND: The question of whether solid malignancies (SMs) are associated with pyoderma gangrenosum (PG) remains to be conclusively answered. OBJECTIVE: To evaluate the risk of SM among patients with PG and the odds of PG after a diagnosis of SM. METHODS: A population-based retrospective cohort study was conducted to study the risk for SM in patients with PG (n = 302) as compared with age-, sex- and ethnicity-matched control subjects (n = 1799). A case-control design was used to estimate the odds of PG in those with a preexisting history of SM. RESULTS: The prevalence of a preexisting SM was comparable in patients with PG and controls (7.5% vs. 8.8%, respectively; P = 0.490). The odds of having PG following a diagnosis of a SM was not statistically increased (OR, 0.85; 95% CI, 0.53-1.36). The incidence of SM was 6.8 (95% CI, 3.5-12.2) and 7.9 (95% CI, 6.1-10.1) per 1000 person-years among patients with PG and controls, respectively. Patients with PG were not more likely to develop SM as compared to controls (HR, 0.86; 95% CI, 0.44-1.69). Patients with a dual diagnosis of PG and SM were older and had more frequent comorbid conditions and increased mortality. CONCLUSIONS: SM is not associated with provoking PG, and patients with PG are not at an increased risk of developing SM. A thorough routine screening for SM in patients with new-onset PG is an unnecessary approach based on the study findings.

Pyoderma Gangrenosum: A Retrospective Study of Clinical Charac-teristics, Comorbidities, Response to Treatment and Mortality Related to Prednisone Dose.

Pyoderma gangrenosum is an uncommon ulcerative neutrophilic dermatosis. Clinical presentation, location and associated diseases are diverse. Treatment of pyoderma gangrenosum includes treating the underlying comorbidity supplemented with topical and/or systemic agents. However, treatment is often challenging. The aim of this study was to explore the diversity of pyoderma gangrenosum and its treatments. A total of 64 patients with pyoderma, at the Department of Dermatology, Aarhus University hospital, Denmark, were included in the study. The patients' records were reviewed over a 6-year period for clinical presentation, associated diseases, treatments and response to treatment, time to mortality after diagnosis and prednisone dose over time. A variety of accompanying comorbidities were found, including a possible association with diabetes. Tumour necrosis α inhibitors were used as third- or fourth-line therapy, but showed the shortest time to remission, and use of prednisone was associated with a higher mortality rate. These findings are discussed in relation to future approaches to treatment of pyoderma gangrenosum.

Estimating the Odds of Ulcerative Colitis-Associated Pyoderma Gangrenosum: A Population-Based Case-Control Study.

BACKGROUND: Ulcerative colitis (UC) is a well-known underlying comorbidity of pyoderma gangrenosum (PG). However, the risk conferred by UC for the subsequent development of PG is yet to be elucidated. We aimed to estimate the magnitude of the association between UC and the subsequent occurrence of PG, which would enable us to assess the odds of PG developing in individuals with a history of UC. METHODS: A population-based case-control study was conducted to compare PG patients (n = 302) and age-, sex- and ethnicity-matched control subjects (n = 1,497) regarding the presence of UC. Logistic regression models were utilized for univariate and multivariate analyses. RESULTS: The prevalence of preexisting UC was greater in patients with PG than in controls (7.3 vs. 0.5%; p < 0.001). A 15-fold increase in the odds of PG in individuals with preexisting UC was observed (OR 14.62, 95% CI 6.45-33.18). The greatest risk of developing PG occurred in the first years following the diagnosis of UC (OR 35.50, 95% CI 4.35-289.60), and decreased thereafter to 10.03 (95% CI 1.83-55.03), 6.69 (95% CI 1.49-30.02), and 10.03 (95% CI 1.83-55.03) at 1-5, 5-10, and 10-15 years after the diagnosis of UC, respectively. This association retained its statistical significance following the adjustment for confounding factors (adjusted OR 10.78, 95% CI 4.55-25.52). Patients with both PG and UC were younger and had a lower prevalence of smoking than the remaining patients with PG. CONCLUSIONS: UC increases the odds of developing PG by 15-fold, with the highest probability of developing PG occurring within the first year after the diagnosis of UC. Patients with UC may be advised to avoid additional precipitating factors for the development of PG.

Characterization and prevalence of extraintestinal manifestations in a cohort of patients with inflammatory intestinal disease in Medellin, Colombia. / Caracterización y prevalencia de manifestaciones extraintestinales en una cohorte de pacientes con enfermedad inflamatoria intestinal en Medellín (Colombia).

INTRODUCTION: Extraintestinal manifestations (EIMs) are frequent in patients with inflammatory bowel disease (IBD). Our objective is to characterize and determine the prevalence of MEIs in our cohort of patients with IBD. PATIENTS AND METHODS: A retrospective study was carried out in adult patients with IBD at the Pablo Tobón Uribe Hospital in Medellín. Colombia. Articular MEIs, primary sclerosing cholangitis (PSC), both ophthalmological and dermatological, were considered. Absolute and relative frequencies were used. The Chi square test of independence was used to compare 2proportions and the odds ratio (OR) was estimated. RESULTS: Our registry has 759 patients with IBD, 544 present UC (71.6%), 200 CD (26.3%) and 15 unclassifiable IBD (1.9%); 177 patients with IBD (23.3%) presented EIMs, 123 of 544 (22.6%) with UC and 53 of 200 (26.5%) with CD (OR: 0.81, 95% CI: 0.55-1.17, P=0.31). Regarding the type of EIMs, the articular ones were the most frequent (13.5%), more in CD than in UC (20.0 vs. 11.3%, OR 1.94, 95% CI: 1.25-3.00, P=0.0037). Patients with IBD and EIMs used more antibodies against tumor necrosis factor (anti-TNFs), compared to those without EIMs (43.5 vs. 18.5%, OR 3.38, 95% CI: 2.31-4.90, P=0.0001). CONCLUSIONS: The prevalence of EIMs in our cohort is high (23.3%) and the most frequent type is joint. Anti-TNFs are most used when IBD and EIMs coexist. Our study provides valuable information on the association of EIMs and IBD in Latin America.

Prevalence of pyoderma gangrenosum and Sweet´s syndrome in inflammatory bowel disease at a tertiary healthcare center.

BACKGROUND AND AIM: dermatological manifestations are normally found in one third of patients with inflammatory bowel disease. In this study, the prevalence, clinical characteristics, intestinal disease activity, and treatment response of neutrophilic dermatoses (pyoderma gangrenosum and Sweet´s syndrome) were determined in patients with inflammatory bowel disease. METHODS: a retrospective, observational study was performed in patients with inflammatory bowel disease and neutrophilic dermatoses between March 2012 and March 2018. RESULTS: of 444 patients analyzed, 10 complied with the inclusion criteria. Seven had pyoderma gangrenosum and three presented Sweet's syndrome; and one patient developed both pathologies. The prevalence of neutrophilic dermatoses was 2.3 % (10/444), comprising 1.6 % with pyoderma gangrenosum and 0.7 % with Sweet's syndrome. Six out of seven patients with pyoderma gangrenosum were female and had ulcerative colitis. The most frequent clinical presentation of pyoderma gangrenosum was the ulcerative subtype. Active moderate-to-severe intestinal disease was found in 71.4 % of patients. Biological therapy was prescribed to three patients with partial response to corticosteroids and persistent intestinal disease activity. This therapy was not indicated for cutaneous manifestations only. Three patients with moderate-to-severe Crohn´s disease presented classical (n = 2) and pustular (n = 1) Sweet's syndrome. A complete response was achieved in all Sweet's syndrome cases treated with corticosteroids. Biological therapy was prescribed to control intestinal disease activity. CONCLUSIONS: pyoderma gangrenosum was the most frequent cutaneous manifestation of neutrophilic dermatoses, predominantly in females with ulcerative colitis, and highly associated with intestinal disease activity. Anti-tumor necrosis factor was effective in patients with partial cutaneous and intestinal disease response.

Quantification of the relationship between pyoderma gangrenosum and Crohn's disease: a population-based case-control study.

BACKGROUND: Although Crohn's disease (CD) is an established underlying disease in pyoderma gangrenosum (PG), studies comparing patients with PG and controls with respect to the presence of CD are lacking. Consequently, the relative risk imposed by CD for the development of PG is yet to be elucidated. OBJECTIVE: The study aims to quantify the magnitude of the association between CD and subsequent development of PG, thus enabling to evaluate the risk of PG with CD. METHODS: A matched case-control study was conducted in Israel comparing PG patients (N = 302) with age-, sex- and ethnicity-matched control subjects (N = 1497) regarding the presence of CD. Logistic regression model was used for multivariate analysis. RESULTS: The prevalence of CD was higher in patients with PG than in control subjects (7.0% vs. 0.3%, respectively; p < .001). There was a 28-fold increase in the odds of PG with CD (OR, 28.08; 95% CI, 9.56-82.41). This association was robust to a sensitivity analysis excluding CD cases diagnosed up to 3 years prior to PG (OR, 30.30; 95% CI, 8.82-104.09), and to a multivariate analysis adjusting for confounding factors (OR, 21.57; 95% CI, 7.20-64.58). The median latency between the diagnosis of CD and the development of PG was 8.08 years. Patients with both PG and CD were younger and had a higher prevalence of smoking when compared to other patients with PG. CONCLUSIONS: CD increases the odds of having PG by 28-folds. Patients with CD should be advised to avoid additional precipitating factors of PG like pathergy and smoking.

Risk factors and treatment outcomes of peristomal pyoderma gangrenosum in patients with inflammatory bowel disease.

BACKGROUND: Insufficient data exist for peristomal pyoderma gangrenosum (PPG), which primarily affects patients with inflammatory bowel disease (IBD). AIMS: To evaluate the risk factors and treatment response of PPG in IBD patients in a real-life cohort. METHODS: Cases of PPG were identified retrospectively using ICD-9/10 codes in patients with IBD who had an ostomy at a tertiary care centre. Disease-specific characteristics were compared between groups with and without PPG, and response to therapy was evaluated in patients with PPG. RESULTS: The cohort included 41 IBD patients with PPG and 123 IBD controls with an ostomy who never developed PPG. Patients with PPG were more likely to be female (76% vs 51%, P = 0.006), had higher BMIs (29.78 ± 0.89 vs 23.53 ± 0.51, P < 0.0001) and had increased usage of pouch belts (97% vs 71%, P = 0.0008) compared to controls. There were no differences in age at surgery (41.76 ± 2.60 vs 43.49 ± 1.50, P = 0.57) or IBD diagnosis (63% vs 54% Crohn's disease, P = 0.28) between PPG and controls. 85% of PPG patients achieved complete resolution with different treatments, including surgery. Complete resolution with topical corticosteroids and calcineurin inhibitors alone were low (14% and 13% respectively). Higher rates of complete resolution were reported with anti-tumour necrosis factor (TNF) agents (63%) and surgical interventions (80%). CONCLUSIONS: Female gender, higher BMI and pouch belts were associated with increased risk of developing PPG. Most PPG cases resolved after treatment with the highest rates of complete resolution seen with anti-TNF agents and surgical intervention.

Pyoderma Gangrenosum in Inflammatory Bowel Disease: A Systematic Review and Meta-Analysis.

BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon but severe extra-intestinal manifestation (EIM) of inflammatory bowel disease (IBD). The incidence and risk factors for PG are disputed. AIMS: To assess the incidence of PG and identify factors associated with PG in IBD patients. METHODS: A search of electronic databases (Ovid and PubMed) was conducted between 1966 and 2019. Studies that calculated the incidence of PG in IBD patient cohorts were included. Patient demographics, IBD subtype, and EIM presence were recorded. A review of our institutional database of 1057 IBD patients was conducted. A multivariate regression model and meta-analysis were conducted to identify risk factors for PG. A random effects model was used to combine the data of included studies. RESULTS: Fourteen studies were included in addition to 1057 IBD patients and 26 PG cases from the Louisville cohort. In total, there were 379 cases of PG in the cumulative cohort of 61,695 IBD patients. The PG incidence in individual studies ranged from 0.4 to 2.6%. In the institutional cohort, ocular EIMs and a permanent stoma were significant risk factors for PG. In the meta-analysis, PG was associated with female gender (RR = 1.328, 95% CI 1.161-1.520), Crohn's disease (RR = 1.193, 95% CI 1.001-1.422), erythema nodosum (RR = 9.281, 95% CI 6.081-14.164), and ocular EIM (RR = 4.55, 95% CI 3.04-6.81). There was study heterogeneity when assessing IBD subtype, ocular, and joint EIMs. CONCLUSIONS: There are conflicting data on the incidence and risk factors for PG. This meta-analysis confirms an association between PG and female gender, Crohn's disease, erythema nodosum, and ocular EIM that have been described in smaller studies.

Pyoderma gangrenosum: a review with special emphasis on Latin America literature.

Pyoderma gangrenosum is a neutrophilic dermatosis characterized by chronic ulcers due to an abnormal immune response. Despite the existence of diagnostic criteria, there is no gold standard for diagnosis or treatment. In Latin America, recognizing and treating pyoderma gangrenosum is even more challenging since skin and soft tissue bacterial and non-bacterial infections are common mimickers. Therefore, this review aims to characterize reported cases of pyoderma gangrenosum in this region in order to assist in the assessment and management of this condition. Brazil, Mexico, Argentina, and Chile are the countries in Latin America that have reported the largest cohort of patients with this disease. The most frequent clinical presentation is the ulcerative form and the most frequently associated conditions are inflammatory bowel diseases, inflammatory arthropaties, and hematologic malignancies. The most common treatment modalities include systemic corticosteroids and cyclosporine. Other reported treatments are methotrexate, dapsone, and cyclophosphamide. Finally, the use of biological therapy is still limited in this region.

Pyoderma gangrenosum: a review with special emphasis on Latin America literature

Abstract Pyoderma gangrenosum is a neutrophilic dermatosis characterized by chronic ulcers due to an abnormal immune response. Despite the existence of diagnostic criteria, there is no gold standard for diagnosis or treatment. In Latin America, recognizing and treating pyoderma gangrenosum is even more challenging since skin and soft tissue bacterial and non-bacterial infections are common mimickers. Therefore, this review aims to characterize reported cases of pyoderma gangrenosum in this region in order to assist in the assessment and management of this condition. Brazil, Mexico, Argentina, and Chile are the countries in Latin America that have reported the largest cohort of patients with this disease. The most frequent clinical presentation is the ulcerative form and the most frequently associated conditions are inflammatory bowel diseases, inflammatory arthropaties, and hematologic malignancies. The most common treatment modalities include systemic corticosteroids and cyclosporine. Other reported treatments are methotrexate, dapsone, and cyclophosphamide. Finally, the use of biological therapy is still limited in this region.

Characterisation and diagnosis of ulcers in inpatient dermatology consultation services: A multi-centre study.

Accurate and prompt diagnosis of skin ulcers is critical to optimise management; however, studies in hospitalised patients are limited. This retrospective review of dermatologic consultations included 272 inpatients with skin ulcers between July 2015 and July 2018 in four U.S. academic hospitals. The median age was 54 years and 45% were male. In 49.3% of the patients, skin ulcers were considered the primary reason for admission. Ulcers of 62% were chronic and 49.6% were located on the lower extremities. Pyoderma gangrenosum (17.3%), infection (12.5%), and exogenous causes (11.8%) were the leading aetiologies; 12% remained diagnostically inconclusive after consultation. Diagnostic agreements pre-dermatology and post-dermatology consult ranged from 0.104 (n = 77, 95% CI 0.051-0.194) to 0.553 (n = 76, 95% CI 0.440-0.659), indicating poor-modest agreement. This study highlights the diagnostic complexity and relative incidences of skin ulcers in the inpatient setting.