-
International Journal of Dermatology Nov 2022Juvenile gangrenous vasculitis of the scrotum (JGVS) is a rare entity with scant reports in the literature. The disease course, treatment, and prevalence have not been... (Review)
Review
Juvenile gangrenous vasculitis of the scrotum (JGVS) is a rare entity with scant reports in the literature. The disease course, treatment, and prevalence have not been well described in the literature. It's hypothesized that JGVS is a variant of pyoderma gangrenosum or a male counterpart of Lipschütz ulcer. This review will analyze the current literature on JGVS and provide a current guide based on the best available data. The initial search of databases yielded 107 studies of which 14 pertained to the topic. The majority of the included studies were case reports (n = 9) reported in Spain. A total of 17 patients were included in the study. The mean age of patients was 22.45 years (range, 13-35 years). The majority of patients presented with multiple, acute, painful, well-circumscribed, round scrotal ulcerations. The majority of patients presented with flu-like symptoms. An increased level of awareness of JGVS diagnosis is now warranted among physicians. Despite the analogies with Lipschütz ulcer, we believe that JGVS is a distinct entity.
Topics: Adolescent; Adult; Female; Gangrene; Genital Diseases, Male; Humans; Male; Pyoderma Gangrenosum; Scrotum; Ulcer; Vasculitis; Vulvar Diseases; Young Adult
PubMed: 35323998
DOI: 10.1111/ijd.16066 -
Annals of Plastic Surgery Aug 2016Pyoderma gangrenosum (PG) is a rare cutaneous disorder that poses a diagnostic challenge in the postoperative period. A systematic literature review was performed to... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare cutaneous disorder that poses a diagnostic challenge in the postoperative period. A systematic literature review was performed to determine distinguishing characteristics of PG in the setting of breast surgery that can facilitate timely diagnosis and appropriate treatment.
METHODS
PubMed, EMBASE, Scopus, and Web of Science databases were systematically searched for articles with cases of PG occurring after breast surgery. Forty-three relevant articles, including 49 case reports, were identified.
RESULTS
PG manifested bilaterally in 30 of 34 cases (88%) in which bilateral surgery was performed. Abdominal wounds were present in 6 of 7 cases in which an abdominal donor site was used for breast reconstruction. Nipples were spared from wound involvement in 33 of 37 cases (89%) in which nipples were present after surgery. Presence of fever was noted in 27 cases (55%) and leukocytosis in 21 cases (43%). A total of 33 patients (67%) underwent wound debridement. Successful medical treatment most commonly involved steroids (41 cases, 84%) and cyclosporine (10 cases, 20%).
CONCLUSIONS
Pertinent clinical features were identified that may aid in timely diagnosis and treatment of PG after breast surgery. Appearance of discrete wounds involving multiple surgical sites that surround but spare the nipples should raise suspicion for PG rather than infection or ischemia, even with concomitant fever and leukocytosis. Wound debridement should be minimized and skin grafting considered only after medical therapy is initiated. Cognizance of these features may enable prompt therapeutic intervention that minimizes morbidity and improves outcomes.
Topics: Female; Humans; Mammaplasty; Mastectomy; Postoperative Complications; Pyoderma Gangrenosum
PubMed: 25003456
DOI: 10.1097/SAP.0000000000000248 -
Aesthetic Plastic Surgery Dec 2021Plastic surgery procedures, including minimally invasive cosmetic procedures, continue to grow in popularity. Although dermatologic complications following plastic... (Review)
Review
PURPOSE
Plastic surgery procedures, including minimally invasive cosmetic procedures, continue to grow in popularity. Although dermatologic complications following plastic surgery procedures are rare, the authors have encountered several of these complications in their practice, including herpes simplex virus (HSV-1) and varicella zoster virus (VZV) infections, pyoderma gangrenosum (PG), contact dermatitis, and suture hypersensitivity. These cases prompted a systematic literature review of dermatologic complications following plastic surgery procedures.
METHODS
The authors conducted a systematic review of PubMed, MEDLINE, EMBASE, Scopus, Web of Science, and the Cochrane Library to identify relevant articles published from 1975 to 2021. Articles were independently reviewed by the authors to determine whether studies met inclusion criteria.
RESULTS
The majority of articles that met inclusion criteria represented level V evidence. The most robust evidence in the literature was for PG, for which there were 63 total studies. Pyoderma gangrenosum was most frequently reported following breast surgery (85.1%), while HSV-1 infections were frequently seen following minimally invasive procedures (84.6%). VZV reactivation was reported after a range of interventions, including pedicled flap surgeries and laser treatments. Other complications, such as suture hypersensitivity, were less frequently reported in the literature, usually as isolated case reports.
CONCLUSIONS
Dermatologic complications represent a rare but serious concern following plastic surgery procedures. While most dermatologic complications resolve with appropriate treatment, sequelae of these conditions can be devastating to the patient's overall outcome. Plastic surgeons performing procedures at a high risk of these complications should recognize the diagnostic criteria to facilitate appropriate treatment.
LEVEL OF EVIDENCE III
This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .
Topics: Bibliometrics; Humans; Mastectomy; Plastic Surgery Procedures; Surgery, Plastic; Surgical Flaps
PubMed: 34231016
DOI: 10.1007/s00266-021-02362-9 -
Journal of the American Academy of... Jun 2024
Review
PubMed: 38906261
DOI: 10.1016/j.jaad.2024.05.086 -
Dermatology (Basel, Switzerland) 2024Pyoderma gangrenosum (PG) is a rare, inflammatory dermatologic disease that, as a diagnosis of exclusion with nonspecific histologic features, is difficult to diagnose....
INTRODUCTION
Pyoderma gangrenosum (PG) is a rare, inflammatory dermatologic disease that, as a diagnosis of exclusion with nonspecific histologic features, is difficult to diagnose. As pharmaceutical interest in potential treatments for PG increases, the need for standardized diagnostic criteria to ensure reproducibility, comparability, and external validity of PG research is required. In this study, we aim to characterize the inclusion and exclusion criteria used in the diagnosis of PG in clinical research studies as well as the eligibility of PG in clinical trials.
METHODS
A systematic review was conducted to characterize the PG inclusion and exclusion criteria in research studies. An additional search of the USA and international clinical trials databases was conducted as well to capture eligibility criteria for PG trials.
RESULTS
Our study revealed a broad range of inclusion and exclusion criteria used to establish the presence or absence of PG. Based on eight distinct categories used to characterize inclusion criteria for research studies, diagnosis by a dermatologist (n = 25, 31.6%), no inclusion criteria listed (n = 21, 26.6%), and clinical and histopathologic features consistent with PG (n = 20, 25.3%) were most common. For current clinical trials, six categories were used to characterize inclusion criteria, of which clinical and histopathologic features consistent with PG (n = 5, 31.3%), identification based on diagnosis of PG (n = 4, 25.0%), and clinical features consistent with PG (n = 3, 18.8%) were the most common.
CONCLUSION
This systematic literature review highlights the range of heterogeneity in diagnostic and eligibility criteria used in PG-directed clinical research and current clinical trials and illustrates the need for the development of consensus guidelines and a rigorous framework to enable high-quality future trials for PG.
Topics: Humans; Pyoderma Gangrenosum; Reproducibility of Results
PubMed: 37879301
DOI: 10.1159/000534750 -
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 -
Frontiers in Immunology 2023This scoping review explores the effectiveness of IL-1 pathway inhibitors in managing PSTPIP1-associated inflammatory diseases (PAID). These diseases are marked by...
INTRODUCTION
This scoping review explores the effectiveness of IL-1 pathway inhibitors in managing PSTPIP1-associated inflammatory diseases (PAID). These diseases are marked by abnormal IL-1 pathway activation due to genetic mutations.
METHODS
Our methodology adhered to a pre-published protocol and involved a thorough search of MEDLINE and EMBASE databases up to February 2022, following the Joanna Briggs Institute Reviewer's Manual and the PRISMA Extension for Scoping Reviews. The review included studies reporting on IL-1 pathway inhibitor use in PAID patients.
RESULTS
From an initial pool of 5,225 articles, 36 studies involving 43 patients were selected. The studies predominantly used observational designs and exhibited diversity in patient demographics, treatment approaches, and outcomes. Anakinra and canakinumab demonstrated promise in treating sterile pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) and PSTPIP1-associated myeloid-related-proteinemia inflammatory (PAMI) syndromes, with scant data on other syndromes. Notably, there was a paucity of information on the adverse effects of these treatments, necessitating cautious interpretation of their safety profile.
CONCLUSION
Current evidence on IL-1 pathway inhibitors for PAID is primarily from observational studies and remains limited. Rigorous research with larger patient cohorts is imperative for more definitive conclusions. Collaborative efforts among specialized research centers and international health initiatives are key to advancing this field.
Topics: Humans; Acne Vulgaris; Adaptor Proteins, Signal Transducing; Antibodies, Monoclonal, Humanized; Arthritis, Infectious; Cytoskeletal Proteins; Interleukin 1 Receptor Antagonist Protein; Interleukin-1
PubMed: 38259483
DOI: 10.3389/fimmu.2023.1339337 -
Journal of Crohn's & Colitis Apr 2024Inflammatory bowel disease (IBD) is associated with various immune mediated disorders including spondylarthritis, pyoderma gangrenosum, primary sclerosing cholangitis...
Inflammatory bowel disease (IBD) is associated with various immune mediated disorders including spondylarthritis, pyoderma gangrenosum, primary sclerosing cholangitis and uveitis. Chronic kidney disease (CKD) is defined by a reduction in kidney function (eGFR less than 60ml/min/1.73m2) and/ or damage markers that are present for at least three months, regardless of the aetiology. Case reports and cohort studies suggest that IBD is associated with CKD. The extent and magnitude of a potential association is unknown. A comprehensive search was conducted in EMBASE, MEDLINE, Web of Science, the Cochrane database, and SCOPUS. Two separate reviewers were involved in the process of article selection and evaluation. Odds ratios were calculated in those papers with a comparison between an IBD population and a non-IBD control population, the Mantel Haenszel test was employed, utilizing a random effect model. The systematic review was registered in PROSPERO (RD42023381927). Fifty-four articles were included in the systematic review. Of these, eight articles included data on prevalence of CKD in IBD patients (n = 102,230) vs. healthy populations (n = 762,430). Of these, diagnosis of CKD was based on ICD codes in five studies vs. on eGFR in three studies. The overall odds ratio of developing CKD in the IBD population is 1.59 (95%CI 1.31-1.93), without any difference between studies using diagnostic coding (OR 1.70 95%CI 1.33-2.19) vs. diagnosis based on eGFR (OR 1.36 95%CI 1.33-1.64). IBD is associated with a clinically meaningful increased CKD prevalence. We provide recommendations on diagnostic evaluation, as well as suggestions for future research.
PubMed: 38584452
DOI: 10.1093/ecco-jcc/jjae049 -
Clinical Gastroenterology and... Jan 2017This systematic review investigated the efficacy and the effectiveness of biologic drugs in extraintestinal manifestations (EIMs) in inflammatory bowel disease (IBD). (Review)
Review
BACKGROUND & AIMS
This systematic review investigated the efficacy and the effectiveness of biologic drugs in extraintestinal manifestations (EIMs) in inflammatory bowel disease (IBD).
METHODS
Literature search was conducted in PubMed, Embase, and Cochrane until October 2015. Main inclusion criteria were adults with IBD, use of a biologic drug, evolution of EIMs, interventional study, or non-interventional study.
RESULTS
Nine interventional studies (2 randomized controlled trials [N = 797], 7 open label trials [N = 1143], and 13 non-interventional studies [N = 914]) were included. Tumor necrosis factor (TNF) antagonists achieved complete response for pyoderma gangrenosum in 21%-25% of patients in interventional studies and in 92%-100% patients in non-interventional studies, with similar results for other cutaneous manifestations such as erythema nodosum or stomatitis. Adalimumab significantly reduced the prevalence of anemia vs placebo after 56 weeks in 1 randomized controlled trial. In 2 non-interventional studies, anti-TNF therapy improved anemia in the short-term (67%) and in the long-term (34%). Complete response after anti-TNF treatment was reported in interventional studies, including arthralgia (reduction in prevalence from 47.1% to 26.8% in the mid-term in 1 open label trial) and arthritis (reduction in prevalence from 8.7% to 2.1% and from 58% to 12.5% in 2 open label trials). Anti-TNFs were beneficial for a majority of patients with ocular manifestations. Infliximab was associated with improved outcomes in bone formation and bone mineral density.
CONCLUSIONS
Anti-TNFs appear to be effective alternatives for certain EIMs associated with IBD including musculoskeletal, cutaneous, and ocular manifestations, and some beneficial effect may be obtained in metabolic bone disease and on hematologic or vascular EIMs.
Topics: Biological Products; Humans; Immunologic Factors; Inflammatory Bowel Diseases; Treatment Outcome; Tumor Necrosis Factor-alpha
PubMed: 27392760
DOI: 10.1016/j.cgh.2016.06.025 -
Dermatologic Therapy Feb 2022Biologic medications are systemic therapeutic options for inflammatory dermatoses. Local forms of administration are less well-studied. To provide a summary of... (Review)
Review
Biologic medications are systemic therapeutic options for inflammatory dermatoses. Local forms of administration are less well-studied. To provide a summary of intralesional (IL) administration of biologics for various non-malignant inflammatory dermatologic conditions reported in the literature. A systematic review was performed in the PubMed and Embase databases from 2000 to 2020. Inclusion criteria included the local use of biologic medications for non-malignant cutaneous conditions. Quality was assessed with the modified Oxford Centre for Evidence-Based Medicine ratings. A total of 19 articles describing the use of 5 biologic medications in 9 dermatologic conditions were identified, comprising 172 patients. Conditions successfully treated with intralesional biologics included pemphigus vulgaris (rituximab), granuloma faciale (rituximab), perianal Crohn's disease (infliximab), lichen sclerosus (adalimumab), and necrobiosis lipoidica (etanercept and infliximab). Intralesional etanercept reduced pruritus associated with keloids. A case report of the use of infliximab for pyoderma gangrenosum did not demonstrate any efficacy. There was no consistent effect noted with treatments for sarcoidosis (infliximab) or cutaneous lymphoid hyperplasia (rituximab). Local administration of biologic medications may offer an additional method of treating refractory inflammatory dermatoses, but further study is needed to develop standardized dosing protocols, clarify efficacy rates, and identify optimal treatment candidates.
Topics: Adalimumab; Biological Products; Etanercept; Humans; Infliximab; Pyoderma Gangrenosum
PubMed: 34825744
DOI: 10.1111/dth.15234