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International Wound Journal Apr 2019Pyoderma gangrenosum (PG) is a rare ulcerative skin disease that presents a therapeutic challenge. Tumour necrosis factor alpha (TNFα) inhibitors have been reported to...
Pyoderma gangrenosum (PG) is a rare ulcerative skin disease that presents a therapeutic challenge. Tumour necrosis factor alpha (TNFα) inhibitors have been reported to successfully control PG. Our aim was to systematically evaluate and compare the clinical effectiveness of TNFα inhibitors in adults with PG. A literature search including databases such as PubMed, Embase, Scopus, and Web of Science was conducted, using search terms related to PG and TNFα inhibitors. Studies and case reports were included if patients were diagnosed with PG, over the age of 18 and administered TNFα inhibitor. A total of 3212 unique citations were identified resulting in 222 articles describing 356 patients being included in our study. The study we report found an 87% (95% CI: 83%-90%) response rate and a 67% (95% CI: 62%-72%) complete response rate to TNFα inhibitors. No statistically significant differences in the response rates (P = 0.6159) or complete response rates (P = 0.0773) to infliximab, adalimumab, and etanercept were found. In our study TNFα inhibitors demonstrated significant effectiveness with response and complete response rates supporting the use of TNFα inhibitors to treat PG in adults. Our study suggests that there is no significant difference in effectiveness among infliximab, adalimumab, and etanercept.
Topics: Adalimumab; Adult; Aged; Aged, 80 and over; Antirheumatic Agents; Etanercept; Female; Humans; Immunologic Factors; Infliximab; Male; Middle Aged; Pyoderma Gangrenosum; Tumor Necrosis Factor-alpha
PubMed: 30604927
DOI: 10.1111/iwj.13067 -
Journal of Cutaneous Medicine and... 2021Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful and ulcerating lesions on the skin. It rarely involves the face and is often... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful and ulcerating lesions on the skin. It rarely involves the face and is often difficult to diagnose. There are few cases reported in the literature of PG involving the face.
AIM
To share our experience with 5 patients in whom the final diagnosis was PG involving the face, and to review the literature.
METHODS
We report a series of 5 patients with a final diagnosis of PG involving the face and reviewed relevant literature. We searched through PubMed andEMBASE using keywords such as "face" and "pyoderma gangrenosum," "blastomycosis-like pyoderma gangrenosum, vegetative pyoderma gangrenosum and granulomatous pyoderma gangrenosum."
RESULTS
We report 5 patients (4 females) with pyoderma gangrenosum involving the face. All 5 had a final diagnosis of superficial granulomatous PG. All cases presented with nonhealing facial ulcer most commonly on cheeks and a common histopathology of mixed inflammatory infiltrates, multinucleated giant cells, and plasma cells with some granulomatous inflammation.
CONCLUSIONS
PG can involve the face, and all 5 of our patients had the superficial granulomatous as the most common form.
Topics: Adolescent; Adult; Aged, 80 and over; Facial Dermatoses; Female; Humans; Male; Middle Aged; Pyoderma Gangrenosum
PubMed: 33541119
DOI: 10.1177/1203475420988864 -
Advances in Wound Care Dec 2020Pyoderma gangrenosum (PG) is a rare neutrophilic ulcerative dermatosis that poses a high burden of morbidity due to underdiagnosis, resistance to therapy, and limited... (Review)
Review
Pyoderma gangrenosum (PG) is a rare neutrophilic ulcerative dermatosis that poses a high burden of morbidity due to underdiagnosis, resistance to therapy, and limited therapeutic options. Optimization of wound care strategies and multimodal anti-inflammatory approaches are necessary to mitigate multiple converging pathways of inflammation leading to delayed healing, which is further complicated by additional factors such as pathergy. PG treatment typically involves reducing inflammation, controlling pain, promoting wound healing, and treating the underlying etiology. Recent advances have been made with regard to targeted therapies for PG with topical, intralesional, and systemic medications. Wound management includes gentle cleansing without sharp debridement, limited topical antibacterial use, and maintenance of a moist environment to promote epithelial migration. Wound dressings and compression therapy, in particular, introduce a wide variety of therapeutic options. Dressings should aim to target the specific PG wound type, depending on the depth and exudative nature of the wound, as well as local secondary factors. Superficial wounds, eschar, exudative wounds, granulating wounds, and colonized wounds are managed with variable approaches to the same underlying principles of pathergy avoidance, moisture balance, and reduction of immunogenic inflammatory stimuli. The importance of compression therapy to decrease edema and overgranulation fits within this treatment paradigm. As each of these treatment modalities offers a complex mixture of advantages and limitations, development of a systematic treatment algorithm in the future can help direct a more tailored path toward wound healing.
Topics: Administration, Topical; Anti-Inflammatory Agents; Compression Bandages; Critical Care; Debridement; Humans; Pyoderma Gangrenosum; Wound Healing
PubMed: 32320358
DOI: 10.1089/wound.2020.1168 -
Internal Medicine (Tokyo, Japan) Feb 2023
Topics: Humans; Pyoderma Gangrenosum; Colitis, Ulcerative; Stomatitis; Gingivitis, Necrotizing Ulcerative; Colectomy
PubMed: 35871595
DOI: 10.2169/internalmedicine.9802-22 -
Frontiers in Immunology 2021This study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China...
OBJECTIVES
This 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.
METHODS
The clinical data and genotype of three Chinese Han patients were carefully documented and studied. We also conducted a systematic literature review on PAPA syndrome.
RESULTS
A 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 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 . 83%), whereas arthritis was less common (50 . 98.1%). Steroid and/or biological agents were effective in most patients.
CONCLUSIONS
The 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.
Topics: Acne Vulgaris; Adaptor Proteins, Signal Transducing; Adult; Arthritis, Infectious; Biological Products; Cytoskeletal Proteins; DNA Mutational Analysis; Female; Genetic Predisposition to Disease; Humans; Male; Middle Aged; Mutation; Phenotype; Predictive Value of Tests; Pyoderma Gangrenosum; Steroids; Treatment Outcome
PubMed: 34745107
DOI: 10.3389/fimmu.2021.735851 -
Acta Dermato-venereologica Apr 2021Pyoderma gangrenosum is an uncommon ulcerative neutrophilic dermatosis. Clinical presentation, location and associated diseases are diverse. Treatment of pyoderma...
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.
Topics: Comorbidity; Humans; Prednisone; Pyoderma Gangrenosum; Retrospective Studies
PubMed: 33686448
DOI: 10.2340/00015555-3776 -
JAMA Dermatology Apr 2018Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis. Current knowledge of this rare disease is limited owing to a lack of validated diagnostic criteria and...
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.
Topics: Adult; Age Factors; Aged; Aged, 80 and over; Arthritis; Arthritis, Rheumatoid; Comorbidity; Female; Humans; Inflammatory Bowel Diseases; Male; Middle Aged; Neoplasms; Paraproteinemias; Polycythemia Vera; Pyoderma Gangrenosum; Retrospective Studies; Spondylitis, Ankylosing; United States
PubMed: 29450453
DOI: 10.1001/jamadermatol.2017.5978 -
JPMA. the Journal of the Pakistan... Jan 2022Pyoderma Gangrenosum (PG) is a rare, debilitating, and painful disease of the skin. Its aetiology and pathophysiology are not well understood. However, it is known that... (Review)
Review
Pyoderma Gangrenosum (PG) is a rare, debilitating, and painful disease of the skin. Its aetiology and pathophysiology are not well understood. However, it is known that PG is not bacterial in origin, as previously believed. A significant number of cases of PG report a phenomenon called pathergy, which is characterized by the appearance of new lesions after the application of trauma to the skin. This represents a unique challenge for surgeons in cases that are refractory to medical therapy. The objective of this study is to review past literature and report a case of PG in a 19-year old woman, who presented with recurrence, after undergoing skin grafting one year back. The patient was referred for split thickness skin grafting (STSG) to reduce the psychological and physical morbidity as a result of this disease.
Topics: Adult; Female; Humans; Pyoderma Gangrenosum; Skin; Skin Transplantation; Young Adult
PubMed: 35099456
DOI: 10.47391/JPMA.11-1109 -
Actas Dermo-sifiliograficas Mar 2012Pyoderma gangrenosum is a condition that is included among the neutrophilic dermatoses. Given its low incidence, few studies have addressed its epidemiology or treatment. (Review)
Review
BACKGROUND
Pyoderma gangrenosum is a condition that is included among the neutrophilic dermatoses. Given its low incidence, few studies have addressed its epidemiology or treatment.
OBJECTIVE
To describe the epidemiological and clinical characteristics of patients with pyoderma gangrenosum along with our experience of treating the condition in a referral hospital in Malaga, Spain.
MATERIAL AND METHODS
A retrospective, observational study was undertaken in the Department of Dermatology at Hospital Clínico Universitario Virgen de la Victoria in Malaga, Spain between January 2000 and December 2009 and included all patients diagnosed with pyoderma gangrenosum.
RESULTS
The incidence of pyoderma gangrenosum in our reference population is 3.26 cases per million inhabitants per year. The most frequent concomitant systemic disease was ulcerative colitis (5 cases, 33%). In 4 patients with that disease, pyoderma gangrenosum appeared during a flare-up. In 80% of cases, patients were not referred to a dermatologist during the initial phase of pyoderma gangrenosum, and most referrals were from gastroenterology or general surgery (4 patients each, 52%).
CONCLUSIONS
Patients with pyoderma gangrenosum are often referred to dermatologists by other specialists after a varying period of time has elapsed without achieving an accurate diagnosis. In these patients, especially those between 20 and 40 years of age, it is essential to rule out concomitant disease. Adalimumab is a good treatment option for pyoderma gangrenosum.
Topics: Adult; Aged; Biopsy; Colitis, Ulcerative; Comorbidity; Delayed Diagnosis; Dermatology; Female; Gastroenterology; General Surgery; Hospitals, University; Humans; Immunosuppressive Agents; Incidence; Male; Middle Aged; Pyoderma Gangrenosum; Referral and Consultation; Retrospective Studies; Skin Ulcer; Spain
PubMed: 21782147
DOI: 10.1016/j.ad.2011.04.010 -
Thorax Apr 2000
Topics: Antibodies, Antineutrophil Cytoplasmic; Biomarkers; Diagnosis, Differential; Granulomatosis with Polyangiitis; Humans; Middle Aged; Pyoderma Gangrenosum
PubMed: 10766647
DOI: 10.1136/thorax.55.4.345d