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Scientific Reports Mar 2023Pyoderma gangrenosum (PG) is a rare inflammatory skin disease classified within the spectrum of neutrophilic dermatoses. The pathophysiology of PG is yet incompletely...
Pyoderma gangrenosum (PG) is a rare inflammatory skin disease classified within the spectrum of neutrophilic dermatoses. The pathophysiology of PG is yet incompletely understood but a prominent role of genetics facilitating immune dysregulation has been proposed. This study investigated the potential contribution of disrupted molecular pathways in determining the susceptibility and clinical severity of PG. Variant Enrichment Analysis, a bioinformatic pipeline applicable for Whole Exome Sequencing data was performed in unrelated PG patients. Eleven patients were enrolled, including 5 with unilesional and 6 with multilesional PG. Fourteen pathways were exclusively enriched in the "multilesional" group, mainly related to immune system (i.e., type I interferon signaling pathway), cell metabolism and structural functions. In the "unilesional" group, nine pathways were found to be exclusively enriched, mostly related to cell signaling and cell metabolism. Genetically altered pathways involved in immune system biology and wound repair appear to be nodal pathogenic drivers in PG pathogenesis.
Topics: Humans; Pyoderma Gangrenosum
PubMed: 36966241
DOI: 10.1038/s41598-023-31914-z -
The American Journal of Case Reports Sep 2022BACKGROUND Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis that can be associated with systemic diseases, such as ulcerative colitis, polyarthritis,... (Review)
Review
BACKGROUND Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis that can be associated with systemic diseases, such as ulcerative colitis, polyarthritis, diabetes mellitus, myelodysplastic syndrome, and/or myeloid leukemia, and is often misdiagnosed as a necrotizing infection. Few reports have described imaging studies of PG; however, necrotizing fasciitis (NF) exhibits distinct imaging characteristics. If deep fascial involvement is not demonstrated on magnetic resonance imaging (MRI), NF is excluded. CASE REPORT We present a case of PG mimicking NF on MRI in a 67-year-old woman with acute myeloblastic leukemia. After undergoing a second cycle of decitabine therapy, she was admitted for pain in her lower left leg. The condition was initially misdiagnosed as NF because MRI findings demonstrated signal intensity in the fascia. MRI revealed fasciitis that exhibited linear fluid signal intensity in the fascia of lower left leg. Despite broad-spectrum antibiotics, the lesion rapidly progressed to a swollen hemorrhagic patch with bullae and an ulcer. Skin biopsy results ultimately led to the diagnosis of PG, based on histopathological findings. The patient was treated with intravenous steroids and regular wound dressing. The skin lesion on the lower left leg exhibited a good response. CONCLUSIONS Despite the presence of a lesion that invaded the fascia on MRI, our patient was diagnosed with PG following a skin biopsy and completely recovered with steroid treatment. To distinguish PG from NF, it is more important to identify the characteristic clinical features than to rely solely on imaging findings.
Topics: Aged; Colitis, Ulcerative; Fasciitis, Necrotizing; Female; Humans; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Pyoderma Gangrenosum
PubMed: 36045564
DOI: 10.12659/AJCR.931734 -
Journal of the American Academy of... Nov 2015
Topics: Adolescent; Adult; Black or African American; Age Factors; Aged; Aged, 80 and over; Comorbidity; Cross-Sectional Studies; Female; Humans; Income; Inflammatory Bowel Diseases; Male; Middle Aged; Pyoderma Gangrenosum; Socioeconomic Factors; United States; White People; Young Adult
PubMed: 26475546
DOI: 10.1016/j.jaad.2015.08.009 -
Wounds : a Compendium of Clinical... Feb 2020Pyoderma gangrenosum (PG) is a neutrophilic dermatosis often associated with autoimmune disease that, despite published literature, still poses diagnostic and...
INTRODUCTION
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis often associated with autoimmune disease that, despite published literature, still poses diagnostic and therapeutic challenges due to its lack of serological and histological markers and wide possibility of differential diagnosis. Although immunosuppressive therapy usually is the first-line treatment, it has a long-lasting, unpredictable treatment course, thus necessitating the need for new treatment options. This report highlights 2 cases of PG ulcers treated with different methods that show a potentially faster healing time, as well as a review of advanced treatment options for PG.
CASE REPORTS
Two men (21 years old and 64 years old) presented with PG ulcers and underwent 2 different treatment courses. The 21-year-old man received negative pressure wound therapy (NPWT) with a nonadhesive, less-invasive, skin-contact interface layer on the polyurethane foam. Stimulated growth of granulation tissue accelerated healing and prepared the wound bed for further epithelization. The second patient, the 64-year-old man, underwent treatment with autologous platelet-rich fibrin, which supplied the ulcer with growth factors and accelerated epithelialization.
CONCLUSIONS
The new possibilities offered by NPWT with nonadhesive foam and regenerative medicine are typically affordable, effective, safe, and painless for the patient. Both methods were noninvasive, did not accelerated the pathergy phenomenon, and showed a potential for faster healing of PG ulcers.
Topics: Humans; Male; Middle Aged; Negative-Pressure Wound Therapy; Platelet-Rich Fibrin; Pyoderma Gangrenosum; Young Adult
PubMed: 32155124
DOI: No ID Found -
Clinics in Dermatology 2021Neutrophilic dermatoses (NDs) are a group of reactive, noninfectious autoinflammatory diseases characterized by (1) infiltration of the epidermis, dermis, and... (Review)
Review
Neutrophilic dermatoses (NDs) are a group of reactive, noninfectious autoinflammatory diseases characterized by (1) infiltration of the epidermis, dermis, and or/hypodermis by neutrophils; (2) their association with distinct diseases (eg, hematologic malignancy and chronic inflammatory diseases); (3) potential extracutaneous involvement; and (4) response to anti-inflammatory drugs, such as corticosteroids, dapsone, colchicine, and novel biologic therapies, such as the anti-interleukin-1 blockade. Although distinct NDs have been described, transitional forms with overlapping features are often identified. These justify a simplified classification of NDs with three major forms: superficial (epidermal or pustular) NDs, dermal (en plaques) NDs, and deep NDs. We review selected or novel variants of NDs, including subcorneal pustular dermatosis, the group of immunoglobulin A neutrophilic dermatoses, amicrobial pustular dermatosis of the folds, and neutrophilic urticarial dermatosis, as well as atypical forms of Sweet syndrome and pyoderma gangrenosum closely mimicking severe infectious diseases. Knowledge of these variants is essential for proper diagnosis, adequate management, and avoidance of a dangerous escalation of therapy, such as unnecessary immunosuppression or extensive surgery.
Topics: Anti-Inflammatory Agents; Dermatitis; Humans; Neutrophils; Pyoderma Gangrenosum; Skin Diseases, Vesiculobullous; Sweet Syndrome
PubMed: 34272020
DOI: 10.1016/j.clindermatol.2020.10.012 -
JAMA Dermatology Mar 2023
Topics: Humans; Pyoderma Gangrenosum; Colitis, Ulcerative
PubMed: 36652229
DOI: 10.1001/jamadermatol.2022.5978 -
Proceedings of the Royal Society of... Apr 1956
Topics: Humans; Pyoderma; Pyoderma Gangrenosum
PubMed: 13322930
DOI: No ID Found -
International Wound Journal Dec 2019Necrotising fasciitis (NF) is mostly a polymicrobial, severe soft tissue infection that progresses rapidly, penetrating through the subcutaneous tissue to the fascial...
Necrotising fasciitis (NF) is mostly a polymicrobial, severe soft tissue infection that progresses rapidly, penetrating through the subcutaneous tissue to the fascial planes and the muscles. The pyoderma gangrenosum (PG), on the other hand, is a rare, rapidly progressive (except for the post-surgical PG), autoinflammatory ulcerative skin and soft tissue condition. In this study, we tried to emphasise the importance of diagnosing the NF as well as the PG. Although these two clinical presentations have some standard features, awareness of different symptoms in detail affect the outcome. Any surgical discipline can face NF or PG and, therefore, should be aware of them to decrease the mortality rate. Forty-five patients with NF and PG who were treated between January 2008 and October 2018 were included in the study and evaluated retrospectively for age, sex, localisation, onset of symptoms and diagnosis, predisposing factors, characteristics of tissue defects, laboratory findings, Laboratory Risk Indicator for Necrotising Fasciitis (LRINEC) scores, isolated microbiological agents, surgical intervention, and mortality rate. Demographic, laboratory, and clinical data were analysed. Among these 45 patients, 14 patients had PG, and 31 patients had NF. The mean age and SD for the NF and PG groups were 50.80 ± 17.67 and 50.78 ± 12.72, respectively. Five patients had rheumatological disorders; four patients had diabetes mellitus (DM) in the PG group. Males had higher risk than females in NF (odds ratio [OR] = 0.077, 95% confidence interval [CI] 0.017-0.34), and females had higher risk in PG (relative risk [RR] = 5). We compared the LRINEC score of NF patients with PG patients. The mean value of this score was 4.53 for PG patients, and 6.06 for NF patients. Fifteen patients (33.3%) had a radiological evaluation. MRI, CT, and USI were used as imaging modalities. Necrotising fasciitis and PG are two distinct entities that are in general difficult to distinguish. Therefore, differential diagnosis and rapid treatment are crucial for lowering the mortality rate.
Topics: Anti-Bacterial Agents; Debridement; Diabetes Mellitus; Diagnosis, Differential; Diagnostic Imaging; Fasciitis, Necrotizing; Female; Glucocorticoids; Humans; Male; Middle Aged; Pyoderma Gangrenosum; Retrospective Studies; Rheumatic Diseases; Skin Transplantation; Surgical Flaps
PubMed: 31418533
DOI: 10.1111/iwj.13196 -
Medicine Sep 2008Erythema nodosum and pyoderma gangrenosum are the most common cutaneous manifestations in inflammatory bowel diseases (IBD). We conducted the current study to assess the...
Erythema nodosum and pyoderma gangrenosum are the most common cutaneous manifestations in inflammatory bowel diseases (IBD). We conducted the current study to assess the cumulative prevalence of erythema nodosum and pyoderma gangrenosum in patients with IBD and to appraise their association with demographic, clinical, and prognostic factors related to IBD. Between 2000 and 2005, data for all patients with IBD at our gastroenterology department were prospectively and systematically collected using a standardized protocol. Among 2402 patients (1521 diagnosed with Crohn disease [63.3%] and 744 with ulcerative colitis [31.0%]), 140 (5.8%) had at least 1 skin manifestation. The most frequent dermatologic symptoms were erythema nodosum (4.0%) and pyoderma gangrenosum (0.75%). In multivariate analyses, erythema nodosum was significantly and independently associated with a diagnosis of Crohn disease (p < 0.001), female sex (p < 0.001), eye and joint involvement (p < 0.001), and pyoderma gangrenosum (p < 0.0001). Among patients with Crohn disease, erythema nodosum was associated with isolated colonic involvement (p = 0.0001). Pyoderma gangrenosum was significantly and independently associated with black African origin (p = 0.003), familial history of ulcerative colitis (p = 0.0005), uninterrupted pancolitis as the initial location of IBD (p = 0.03), permanent stoma (p = 0.002), eye involvement (p = 0.001), and erythema nodosum (p < 0.0001). It is noteworthy that the association between pyoderma gangrenosum and permanent stoma persisted after exclusion of patients with peristomal pyoderma gangrenosum (p = 0.07). In conclusion, neither erythema nodosum nor pyoderma gangrenosum was significantly associated with the severity criteria in IBD; however, their occurrence may reflect a peculiar phenotype among affected patients.
Topics: Adult; Cohort Studies; Erythema Nodosum; Female; Humans; Inflammatory Bowel Diseases; Male; Multivariate Analysis; Prevalence; Prospective Studies; Pyoderma Gangrenosum; Risk Factors
PubMed: 18794711
DOI: 10.1097/MD.0b013e318187cc9c -
Wounds : a Compendium of Clinical... Jun 2022Pyoderma gangrenosum (PG) is a rare, chronic, inflammatory, and ulcerative condition that often affects the lower extremities. Laboratory markers such as...
INTRODUCTION
Pyoderma gangrenosum (PG) is a rare, chronic, inflammatory, and ulcerative condition that often affects the lower extremities. Laboratory markers such as neutrophil-to-lymphocyte ratio, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) level may have prognostic utility in the management of ulcerative or inflammatory conditions.
OBJECTIVE
This exploratory analysis investigated the relationship between readily available clinical factors and target ulcer healing within 6 months from the first visit.
METHODS
Two logistic regression models were fit-one focused on ESR as a primary predictor adjusted for age and hemoglobin level, and the other focused on CRP level adjusted for body mass index (BMI) and smoking history.
RESULTS
The odds of wound healing at 6 months for patients with abnormal CRP was 0.19 times the odds of healing (95% CI, 0.03-0.77) for those with normal CRP (<10 mg/L), after accounting for BMI and smoking. Similarly, after accounting for age and hemoglobin, those with high ESR experienced 2.81 times the odds of healing (95% CI, 0.69-14.58) at 6 months compared with patients with normal ESR.
CONCLUSIONS
After adjustment, an increased CRP level was associated with lower odds of healing; however, further research is warranted to investigate its prognostic utility. The relationship between ESR and odds of healing should be interpreted with caution because the wide 95% CI for the adjusted odds ratio reflects marked uncertainty in the estimate. This research brief may inform investigations into inflammatory markers as possible prognostic markers for wound healing in patients with PG.
Topics: Biomarkers; Blood Sedimentation; C-Reactive Protein; Humans; Inflammation; Pyoderma Gangrenosum; Retrospective Studies; Ulcer; Wound Healing
PubMed: 35767845
DOI: 10.25270/wnds/2022.0408