Pyoderma Gangrenosum with an Underlying Leukocyte Adhesion Deficiency Type 1 (LAD-1) and Pregnancy in the Shade of COVID-19 Epidemic: A Patient and Physician Experience.
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ABSTRACT:
SUBMITTER: Opalinska A
PROVIDER: S-EPMC7939102 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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.
| S-EPMC8397920 | biostudies-literature
Project description:ObjectivesThis study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China and provide an up-to-date literature review.MethodsThe clinical data and genotype of three Chinese Han patients were carefully documented and studied. We also conducted a systematic literature review on PAPA syndrome.ResultsA total of three patients were diagnosed with PAPA syndrome at our center from 2018 to 2020. Arthritis was observed in all three patients, while pyoderma gangrenosum (PG) was found in two patients and acne in one patient. Other manifestations included pathergy reaction, intermittent fever, oral ulcer, keratitis, proteinuria, and hematuria. The PSTPIP1 A230T mutation was identified in two patients, and a novel Y119C variation was revealed in a sporadic patient. A total of 76 patients with PAPA syndrome reported in 29 articles were included in our literature review. The classical triad of arthritis, PG, and acne was visible in only 16 (25.4%) patients, while 24 (38.1%) exhibited only one major symptom. Skin lesions were more commonly seen in patients with adult-onset disease than those with childhood-onset disease (100 vs. 83%), whereas arthritis was less common (50 vs. 98.1%). Steroid and/or biological agents were effective in most patients.ConclusionsThe rarity and phenotypic heterogeneity associated with PAPA syndrome make the diagnosis a huge challenge to physicians, especially in adult patients. A significant portion of patients did not exhibit the full spectrum of the classical triad. Accordingly, gene testing is critically helpful for diagnosis.
| S-EPMC8569796 | biostudies-literature
Project description:BACKGROUND:Whether the underlying disease affects the outcomes in pyoderma gangrenosum (PG) is unclear. OBJECTIVES:To determine the impact of comorbid disease associations and concomitant procedural treatments on patient outcomes in hospitalizations of patients with PG. METHODS:A cross-sectional analysis of the National Inpatient Sample for hospitalizations of patients with PG from the years 2002 to 2011, analyzing in-hospital mortality rate and health care resource utilization. RESULTS:Inflammatory bowel disease was the most frequent comorbid association, followed by inflammatory arthritis, hematologic malignancies/dyscrasia, and vasculitis. Multivariable modeling showed that vasculitis and hematologic malignancy/dyscrasia, when compared with inflammatory bowel disease, were associated with a 4-fold to 6-fold increased risk of in-hospital mortality and increasing health care resource utilization. Inpatient procedural interventions, including skin grafts, biopsies, and debridement, did not affect mortality and were associated with an increased length of stay. LIMITATIONS:The database does not account for outpatient follow-up; additionally, there was a low rate of coded comorbid conditions. CONCLUSIONS:Comprehensive evaluation to determine the underlying comorbidity for patients with PG is important for patient risk stratification.
| S-EPMC6086769 | biostudies-literature
Project description:Leukocyte adhesion deficiencies are rare clinical syndromes of impaired host defense that provide novel insights into regulation of immune and inflammatory responses. Leukocyte adhesion deficiency (LAD)-I variant (LAD-Iv), also called LAD-III, is a unique disorder in which inside-out signaling of ??, ??, and ?? integrins on leukocytes and platelets is disrupted, leading to impaired cellular adhesion, recurrent infections, and bleeding. We originally reported the second patient with this disorder to be identified and characterized the adhesive deficiencies and functional phenotype of this subject's leukocytes. Here, we show that the molecular defect in this index subject is a new mutation in FERMT3 (KINDLIN-3) which encodes KINDLIN-3, a cytoskeletal protein that interacts with the cytoplasmic tails of ??, ??, and ?? integrins and is required for inside-out and outside-in signaling of these heterodimers. We also report clinical features and previously unrecognized defects in cells from a new patient, a sibling of the original subject that we described who carries the same FERMT3 mutation. Mutations in FERMT3 have now been shown to be the basis for LAD-Iv/LAD-III in each of the four original patients or families that established this syndrome, including the family that we describe.
| S-EPMC3564639 | biostudies-literature
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.
| S-EPMC7370553 | biostudies-literature
Project description: Not available
| S-EPMC7489266 | biostudies-literature
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.
| S-EPMC4432544 | biostudies-other
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.
| S-EPMC8266709 | biostudies-literature
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.
| S-EPMC8453546 | biostudies-literature
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.
| S-EPMC1288212 | biostudies-literature