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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 -
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 -
Journal of the American Academy of... Sep 2022
Topics: Humans; Pyoderma Gangrenosum; Rituximab; Treatment Outcome
PubMed: 34942295
DOI: 10.1016/j.jaad.2021.12.028 -
Frontiers in Immunology 2022Hidradenitis suppurativa were associated with comorbidities in various organ systems. Inflammatory dermatological diseases such as pyoderma gangrenosum were reported to... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Hidradenitis suppurativa were associated with comorbidities in various organ systems. Inflammatory dermatological diseases such as pyoderma gangrenosum were reported to be associated with hidradenitis suppurativa. Nevertheless, as for the association between hidradenitis suppurativa and psoriasis, evidences were insufficient. In many studies, the association between psoriasis and hidradenitis suppurativa has been reported. However, some evidence seems to be controversial. The purpose of the systematic review and meta-analysis was to assess whether there was significant association between HS and psoriasis.
METHODS
On June 01, 2022, we appraised 2,795 articles from databases including PubMed, Web of Science and Embase. Search syntaxes were based on 'hidradenitis suppurativa' or 'acne inversa' with "psoriasis", "comorbidities" or 'epidemiology'. Synonyms were determined based on MeSH terms and Emtree. Observational results that evaluated the odds ratio for people with hidradenitis suppurativa who had psoriasis were extracted for qualitative synthesis.
RESULTS
After the selection process of the initial 2,795 studies, ten observational studies, including 3 cohort studies, 1 case-control study, and 6 cross-sectional studies, were extracted for critical appraisal. Based on the integration of 7 studies (with more than 560,000 participants included), people with hidradenitis suppurativa had a higher risk of having psoriasis, with a 2.67-fold risk (95% CI, 1.84, 3.87). The association remained in the sensitivity analyses utilizing strict adjustment models. In the analysis that only included studies with a similar study design and adjustments in obesity-related factors, the risk of people with hidradenitis suppurativa having psoriasis was 3.24 (95% CI, 2.27, 4.62). In male patients with HS, the risk of having psoriasis was 4.30-fold higher than male patients without HS (95% CI, 2.37, 7.78). Likewise, in an analysis including 3 cross-sectional studies, the risk of female HS patients having psoriasis was 3.94-fold higher than female HS-free patients (95% CI, 2.34, 6.63).
CONCLUSIONS
The co-occurrence of hidradenitis suppurativa and psoriasis can greatly increase the burden of the disease. Psoriasis could be one of the critical comorbidities of hidradenitis suppurativa and should be recommended for future screening and follow up. The association between the two diseases should be kept in mind in managing hidradenitis suppurativa patients. More prospective studies are needed to establish the true magnitude of the association between psoriasis and hidradenitis suppurativa.
Topics: Humans; Male; Female; Cross-Sectional Studies; Case-Control Studies; Hidradenitis Suppurativa; Psoriasis; Comorbidity
PubMed: 36532043
DOI: 10.3389/fimmu.2022.1033844 -
Journal of the American Academy of... Jun 2020Pyoderma gangrenosum (PG) is a devastating neutrophilic dermatosis that may be associated with trauma or systemic diseases. The associations, characteristics, and...
BACKGROUND
Pyoderma gangrenosum (PG) is a devastating neutrophilic dermatosis that may be associated with trauma or systemic diseases. The associations, characteristics, and temporal relationship of PG with hematologic malignancies are not well understood.
OBJECTIVE
We performed a systematic review of PG associated with hematologic malignancies using data from case reports, case series, and retrospective studies.
METHODS
We searched MEDLINE, EMBASE, Scopus, and Web of Science from each database's inception to December 12, 2018. Two reviewers independently selected studies and extracted data.
RESULTS
Two hundred seventy-nine publications met the inclusion criteria (340 cases). Myelodysplastic syndrome (MDS) was the most commonly reported hematologic malignancy associated with PG, followed by monoclonal gammopathy of undetermined significance and acute myeloid leukemia. The mean age of patients was 56.5 years, with males being more common. There was a predominance of the ulcerative PG subtype and multifocal distributions across all hematologic malignancies. The majority of MDS cases preceded PG, which was reversed for MGUS.
LIMITATIONS
The data were limited by reporting bias because PG subtypes rely on the rendered diagnosis reported. In addition, the classification for hematologic malignancies has evolved since 1978.
CONCLUSION
Patients with PG should be evaluated for hematologic malignancies, with MDS being the most common.
Topics: Hematologic Neoplasms; Humans; Leukemia, Myeloid, Acute; Monoclonal Gammopathy of Undetermined Significance; Myelodysplastic Syndromes; Pyoderma Gangrenosum
PubMed: 31560977
DOI: 10.1016/j.jaad.2019.09.032 -
Journal of Plastic, Reconstructive &... Jan 2024Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis that can develop at a surgical site. Diagnosis can be challenging at its presentation causing... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis that can develop at a surgical site. Diagnosis can be challenging at its presentation causing delays in appropriate treatment. The aim of this study is to review the current literature as well as to describe the clinical presentation, diagnostic pathway, and treatment of PG after reduction mammaplasty in order to define a standardized multidisciplinary diagnostic and therapeutic approach. In the future, this may ease early identification and prompt treatment, and eventually minimize severe morbidity and long-term sequelae.
METHODS
The entire PubMed/Medline database was screened following the PRISMA guidelines to identify studies describing PG that have occurred after reduction mammoplasty.
RESULTS
Twenty-eight articles including 31 patients reported a PG after breast reduction surgery between January 1988 and March 2022. Twenty-one (68%) patients presented with skin ulcerations, 14 (45%) with erythema, and 5 (16%) with vesicles. Out of the 30 cases that underwent bilateral surgery, 18 (60%) developed PG bilaterally. In 12 out of 31 patients, nipple-areolar complex (NAC) involvement was evaluated, though in 10 patients (83%) the NAC was spared. Of the 20 patients (65%) who underwent skin biopsies for histopathological examination, 18 (90%) showed neutrophilic infiltration of the dermal layers. All 31 patients (100%) showed rapid clinical improvement after the introduction of immunosuppressive therapy.
CONCLUSIONS
PG can result in devastating skin alterations also after reduction mammoplasty, if misdiagnosed. However, it presents with constant yet unspecific local and general signs and symptoms that can be recognized to early initiate an appropriate pharmacological treatment.
Topics: Female; Humans; Pyoderma Gangrenosum; Mammaplasty; Skin; Mastectomy; Immunosuppression Therapy
PubMed: 38118291
DOI: 10.1016/j.bjps.2023.11.041