Project description:Pyoderma gangrenosum is a neutrophilic dermatosis, which mimics both infection and necrotizing fasciitis, that can present after surgical interventions. We present the case of a 62-year-old male who underwent one-stage bilateral total knee arthroplasty. Nine days after the surgery, he presented with wound breakdown, high fever, and elevated white blood cell count. Repeated debridement was performed, and empiric antibiotics were given. All tissue cultures and aspirates remained negative throughout treatment course, and the patient remained unresponsive to therapy. The patient was eventually diagnosed with pyoderma gangrenosum after infectious etiologies were ruled out and after a skin biopsy and dermatologic consultation. His condition rapidly improved after treatment with corticosteroids, and soft-tissue defects were repaired with skin substitute and full-thickness skin grafting. In patients with aseptic wound breakdown after total knee arthroplasty, pyoderma gangrenosum is a rare but devastating complication and should be considered.
Project description:Pyoderma gangrenosum is a challenging disease to manage, due in part to the lack of approved treatment therapies. Recently, the emergence of biologic agents has expanded treatment options, with tumour necrosis factor alpha inhibitors being the best supported in the literature. In our report, we present a 50-year-old female with pyoderma gangrenosum who was successfully treated with the anti-interleukin-17 biologic agent, secukinumab, after failing other systemic therapies.
Project description:INTRODUCTION: The cocaine is obtained from the leaves of the coca (Erythroxylon coca). It can be used in many ways, but the most common is the drug inhalation. The Cocaine also causes vasoconstriction at nasal mucous membrane and its chronic use can cause necrosis and nasal septum perforation. Pyoderma gangrenosum is an uncommon idiopathic disease characterized by ulcerations, usually observed on the legs. Its diagnosis is most common an exclusion of others diseases. So far, there is no specific treatment based on evidence by randomized controlled trials. OBJECTIVE: Describe the rare association between Pyoderma gangrenosum and cocaine. CASE REPORT: E. A., 27-year-old woman with destruction of nasal septum and palate who has been using a big amount of cocaine, been necessary note the difference from which disease cause de damage. Final Comments: Also there are only three cases of Pyoderma gangrenosum complicated with nasal septum perforation in cocaine users.
Project description:The coexistence of pyoderma gangrenosum (PG) and chronic renal comorbidities has been reported anecdotally. We aimed to assess the bidirectional association between PG and the following chronic renal comorbidities: chronic renal failure (CRF), dialysis, kidney transplantation (KT), and other kidney diseases (OKD). That is to evaluate (i) the risk of the aforementioned diseases among patients with PG (ii) and the odds of PG after a diagnosis of renal comorbidities. A population-based retrospective cohort study was conducted comparing PG patients (n=302) with age-, sex-, and ethnicity-matched control subjects (n=1497) with regard to incident cases of renal comorbidities. A case-control design was additionally adopted to estimate the odds of PG in those with a preexisting history of renal comorbidities. Adjusted hazard ratios (HRs) and adjusted odds ratios (ORs) were estimated by Cox regression and logistic regression, respectively. Patients with PG demonstrated an increased risk of CRF (adjusted HR, 3.68; 95% CI, 2.72-5.97), dialysis (adjusted HR, 27.79; 95% CI, 3.24-238.14), and OKD (adjusted HR, 2.71; 95% CI, 1.55-4.74). In addition, the odds of PG were increased after the diagnosis of CRF (adjusted OR, 2.34; 95% CI, 1.33-4.11), KT (adjusted OR, 5.03; 95% CI, 1.01-25.12), and OKD (adjusted OR, 1.69; 95% CI, 1.04-2.74). Patients with a dual diagnosis of PG and renal diseases presented with PG at an older age and had a higher prevalence of comorbid conditions. In conclusion, a bidirectional association exists between PG and chronic renal conditions. Awareness of this comorbidity may be of benefit for physicians managing patients with PG.
Project description:Pyoderma gangrenosum (PG) is a neutrophilic dermatose (ND) characterized by a dense neutrophilic infiltrate in the affected tissue. Neutrophil extracellular traps (NETs) are web-like structures released by neutrophils and composed of cytosolic and granule proteins assembled on a scaffold of decondensed chromatin. Very little is known about the role of NETosis in PG. Here, we assessed the possible implication of NETosis in the pathogenesis of PG by investigating the NETosis in the ulcers of 26 PG patients. We demonstrated that neutrophils in the PG skin lesions undergo an aberrant level of NETosis in 100% of the analysed cases (N = 26). All control and abscess biopsies were instead negative for the NETosis. In addition, neutrophils from peripheral blood of PG patients showed a significantly higher rate of spontaneous, but not induced, NETosis. Overall, this study suggests that the NETosis may contribute to systemic inflammation and tissue destruction in PG, thus representing a possible novel therapeutic target.
Project description:Pyoderma gangrenosum, cystic acne, and aseptic arthritis are clinically distinct disorders within the broad class of inflammatory diseases. Although this triad of symptoms is rarely observed in a single patient, a three-generation kindred with autosomal-dominant transmission of these three disorders has been reported as "PAPA syndrome" (MIM 604416). We report mapping of a disease locus for familial pyoderma gangrenosum-acne-arthritis to the long arm of chromosome 15 (maximum two-point LOD score, 5.83; recombination fraction [straight theta] 0 at locus D15S206). Under the assumption of complete penetrance, haplotype analysis of recombination events defined a disease interval of 10 cM, between D15S1023 and D15S979. Successful identification of a single disease locus for this syndrome suggests that these clinically distinct disorders may share a genetic etiology. These data further indicate the role of genes outside the major histocompatibility locus in inflammatory disease.
Project description:Pyoderma gangrenosum (PG) is a relatively uncommon inflammatory skin disorder that is characterized by rapid onset, ulcerative lesions, and often triggered by trauma or surgery. Although rare, PG of the breast has been well described in the plastic surgery literature, most often reported following breast reductions and reconstructions. The authors present a case of PG that developed in a 56-year-old patient, with a history of essential thrombocytosis, following mastopexy. Her significant full-thickness skin loss was successfully treated with steroids and then reconstructed via serial applications of porcine placental extracellular matrix grafts. Her wounds were completely healed in 3.5 months for the left, and 5 months for the right breast. To the best of the author's knowledge, this is the first published case in which placental grafts have been successfully used to reconstruct pyoderma wounds of the breast. Given their advantageous scarring and lack of donor-site morbidity, placental grafts should be considered for all breast surgery patients afflicted by PG.Level of evidence 5
Project description:Pyoderma gangrenosum is a rare inflammatory neutrophilic disorder with no uniformly effective therapy and limited high-level evidence. Common therapies include immunosuppressive and immunomodulating agents. There exist several case series using small molecules as treatment modalities. Here, we report a case of a 78-year-old female with a diagnosis of pyoderma gangrenosum and metastatic high-grade serous carcinoma of the ovary who was treated with Baricitinib 4 mg daily in combination with a tapering course of prednisone after failing other conventional therapies including systemic corticosteroids, colchicine, and intravenous immunoglobulin.
Project description:Importance:Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis. Current knowledge of this rare disease is limited owing to a lack of validated diagnostic criteria and large population studies. Objective:To evaluate the association of age with the clinical presentation and comorbidities of pyoderma gangrenosum. Design, Setting, and Participants:This was a multicenter retrospective cohort study performed at tertiary academic referral centers in urban settings. Adults (≥18 years) who were evaluated and diagnosed as having pyoderma gangrenosum at the Brigham and Women's and Massachusetts General Hospitals from 2000 to 2015 and the University of Pennsylvania Health System from 2006 to 2016 were included. Main Outcomes and Measures:Patient demographics, clinical features, medical comorbidities, and treatment. Results:Of the 356 validated cases of pyoderma gangrenosum included in the study, 267 (75%) were women and 284 (84.8%) were white. The mean (SD) age at presentation was 51.6 (17.7) years. Pathergy was recorded in 100 patients (28.1%). A total of 238 patients (66.9%) had associated medical comorbidities: inflammatory bowel disease in 146 patients (41.0%); inflammatory arthritis in 73 patients (20.5%); solid organ malignant neoplasms in 23 patients (6.5%); hematologic malignant neoplasms in 21 patients (5.9%); and hematologic disorders, specifically monoclonal gammopathy of undetermined significance, myelodysplastic syndrome, and polycythemia vera in 17 patients (4.8%). When stratified by age, pathergy was more common in patients 65 years or older (36.3% vs 24.3%; P = .02). Inflammatory bowel disease was the only medical comorbidity that was more common in patients younger than 65 years (47.7% vs 26.6%; P < .001), while a number of medical comorbidities were more common in those 65 years or older, including rheumatoid arthritis (13.3% vs 6.2%; P = .03), ankylosing spondylitis (1.8% vs 0%; P = .04), solid organ malignant neoplasms (13.3% vs 3.3%; P < .001), hematologic malignant neoplasms (9.7% vs 4.1%; P = .04), and the aforementioned hematologic disorders (10.6% vs 2.1%; P < .001). Conclusions and Relevance:Although clinical presentation in this large cohort was similar between different age groups, disease associations varied by age. The findings of this study may allow for a more focused, age-specific evaluation of patients with pyoderma gangrenosum.