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International Journal of Dermatology May 2017Pyoderma gangrenosum (PG) is a sterile neutrophilic disorder that rarely affects children. Clinical, epidemiological, and therapeutic data on pediatric PG is poor as... (Review)
Review
Pyoderma gangrenosum (PG) is a sterile neutrophilic disorder that rarely affects children. Clinical, epidemiological, and therapeutic data on pediatric PG is poor as there are many newly reported associated diseases and drugs. This paper aims to review all recent available data on pediatric PG. A systematic review of the literature was conducted using Embase, Medline, and Cochrane databases. A total of 132 articles were included in the review. The most commonly reported underlying diseases in pediatric PG are inflammatory bowel diseases followed by hematologic disorders, vasculitis, immune deficiencies and Pyogenic Arthritis, Pyoderma gangrenosum and Acne (PAPA) syndrome. More than half of the cases occur with no underlying disease. The most frequently reported clinical presentation is multiple disseminated ulcers. Treatment should be tailored according to the underlying etiology. It includes systemic steroids, corticosteroid sparing agents such as dapsone and cyclosporine, and TNF-alpha inhibitors such as adalimumab and infliximab. Response to treatment is high with cure rates reaching 90%. A high index of suspicion and a thorough workup are mandatory in the management of pediatric PG.
Topics: Acne Vulgaris; Adolescent; Arthritis, Infectious; Child; Child, Preschool; Hematologic Diseases; Humans; Immunologic Deficiency Syndromes; Infant; Infant, Newborn; Inflammatory Bowel Diseases; Pyoderma Gangrenosum; Vasculitis
PubMed: 28233293
DOI: 10.1111/ijd.13584 -
Journal of the American Academy of... Mar 2017Pyoderma gangrenosum (PG) is a rare, ulcerative cutaneous disorder. Ophthalmic involvement in PG is atypical, but can have devastating consequences. (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare, ulcerative cutaneous disorder. Ophthalmic involvement in PG is atypical, but can have devastating consequences.
OBJECTIVE
We sought to characterize ocular PG to allow for earlier diagnosis and therapy. To our knowledge, this is the first systematic review summarizing this clinical variant.
METHODS
A systematic review was conducted using PubMed and Web of Science. Data were extracted and studies were qualitatively assessed and analyzed.
RESULTS
We identified all 34 cases of PG involving the eye and periorbital area, and categorized them into 4 different subtypes. Common presenting signs include ulceration, peripheral ulcerative keratitis, and decreased visual acuity. Although it is often difficult to biopsy ocular PG, histologic features are nonspecific. Combined therapy using corticosteroids and further surgical reconstruction as needed is the mainstay of treatment. Cases of the eye/orbit in particular should be treated aggressively, as these are more likely to relapse compared with cases of the periorbital area.
LIMITATIONS
Use of case reports, paucity of ocular PG cases, and heterogeneity of studies are limitations.
CONCLUSION
PG should be considered in the differential diagnosis of ulceration of ocular/periocular tissues. An aggressive, early, multimodal treatment strategy should be used to prevent relapse, especially in cases of the eye/orbit.
Topics: Comorbidity; Diagnosis, Differential; Eye Diseases; Humans; Pyoderma Gangrenosum
PubMed: 27836332
DOI: 10.1016/j.jaad.2016.08.049 -
The Thoracic and Cardiovascular Surgeon Jan 2023Pyoderma gangrenosum after cardiac surgery is a rare, noninfectious ulcerating skin disease mimicking sternal wound infection.
BACKGROUND
Pyoderma gangrenosum after cardiac surgery is a rare, noninfectious ulcerating skin disease mimicking sternal wound infection.
METHODS
A systematic search of literature for pyoderma gangrenosum complicating cases of cardiac surgery was conducted between September 1985 and September 2020 on PubMed and Cochrane databases. A systematic review and detailed overview of clinical presentation, diagnostic, treatment, and outcome is provided.
RESULTS
A total of 15 studies enclosing 15 patients suffering from pyoderma gangrenosum following cardiac surgery were identified. Onset of symptoms was observed after a median of 5 days. Patients were predominantly male (81.3%) with a median age of 64 years. Typical clinical presentation mimicked sternal site infection, mainly by means of mediastinitis. Specific signs were rapid progression, erythematous to violaceous color of the wound border, accompanied by unspecific symptoms including fever, malaise, and severe pain. Additionally, pathergy (development of ulcers at the sites of minor cutaneous trauma) was reported frequently. Biopsy is mandatory with a cutaneous neutrophilic inflammation confirming the diagnosis. Initial treatment mostly (75.0% of reported cases) was misled, addressing suspicion of surgical site infection. After correct diagnosis, the treatment was switched to an immunosuppressive therapy. Full sternal wound closure took between 5 weeks and 5 months. Reported case mortality was 12.5% in actually low-risk surgeries.
CONCLUSION
Despite pyoderma gangrenosum has typical signs, it remains an exclusion diagnosis. The treatment is completely opposite to the main differential diagnosis-the typical surgical site infection. Knowledge about diagnosis and treatment is essential in the context of avoiding fatal mistreatment.
Topics: Humans; Male; Middle Aged; Female; Pyoderma Gangrenosum; Surgical Wound Infection; Treatment Outcome; Cardiac Surgical Procedures
PubMed: 34894635
DOI: 10.1055/s-0041-1735960 -
Journal of Translational Autoimmunity 2020In the last decade, new scientific findings significantly improved our understanding of the molecular pathogenesis of autoinflammation and have resulted in the... (Review)
Review
In the last decade, new scientific findings significantly improved our understanding of the molecular pathogenesis of autoinflammation and have resulted in the identification and definition of several pyoderma gangrenosum-associated autoinflammatory syndromes (PGAAIS) as new and distinct clinical entities. These different clinical entities include PAPA (pyogenic arthritis, pyoderma gangrenosum and acne conglobata), PASH (pyoderma gangrenosum, acne and suppurative hidradenitis), PAPASH (pyoderma gangrenosum, acne, suppurative hidradenitis and pyogenic arthritis), PsAPASH (pyoderma gangrenosum, acne, suppurative hidradenitis and psoriatic arthritis), PASS (pyoderma gangrenosum, acne conglobata, suppurative hidradenitis, and axial spondyloarthritis) and PAC (pyoderma gangrenosum, acne and ulcerative colitis), which can be distinguished by their clinical presentation and the presence or absence of mutations in several genes, such as the genes encoding proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1), nicastrin (NCSTN), Mediterranean fever (MEFV) and nucleotide-binding oligomerization domain-containing protein (NOD). In this systematic review, we summarize the present knowledge of this rapidly developing hot topic and provide a guide to enable the easy diagnosis of these syndromes in everyday clinical practice. Moreover, we report a rare case of PASS syndrome demonstrating successful treatment with adalimumab and another case of a previously unreported combination of symptoms, including psoriatic arthritis, pyoderma gangrenosum, suppurative hidradenitis and Crohn's disease (newly coined PsAPSC), as examples. Because of the identification of similar genetic and pathogenic mechanisms of PGAAIS, we think the wide variety of seemingly different syndromes may represent distinct phenotypes of one disease.
PubMed: 33305249
DOI: 10.1016/j.jtauto.2020.100071 -
Dermatologic Therapy Jun 2022Cyclosporine-A (Cyc-A) was initially prescribed as systemic therapy for patients receiving solid organ transplants or in patients with graft versus host disease (GVHD).... (Review)
Review
Cyclosporine-A (Cyc-A) was initially prescribed as systemic therapy for patients receiving solid organ transplants or in patients with graft versus host disease (GVHD). Topical Cyc-A is an ideal form of cyclosporine in the treatment of mucocutaneous disorders as it causes fewer systemic side effects and has more stable results than steroids; however, poor absorption through the skin makes the development of new formulations necessary to improve skin permeability. The aim of this study was to evaluate the efficacy and safety of topical Cyc-A in different dermatological conditions. A thorough systematic review was performed on PubMed/Medline, Embase, Scopus, and Web of Science databases as well as Google Scholar, and relevant studies from 2000 until January 3, 2022, were selected. The study was conducted according to the Preferred Reporting Items for Systematic reviews and Meta-Analysis (PRISMA). Topical Cyc-A was observed to be an effective medication in the treatment of oral lichen planus, psoriasis, burning mouth syndrome, Pyoderma Gangrenosum, and Zoon's balanitis. Adverse side effects such as dysphagia, burning sensation, lips swelling, and gastrointestinal upset were reported following Cyc-A mouthwash use, whereas mild erythema, dryness, and fissuring of the skin were observed following the Cyc-A lipogel application. Topical Cyc-A was found to be a good alternative to traditional treatment regimens for immune-mediated mucocutaneous conditions. Cyc-A can be considered as a safe and efficient option in cases of long-term treatment as it does not have the same adverse effects of long-term steroids.
Topics: Cyclosporine; Dermatology; Humans; Lichen Planus, Oral; Male; Psoriasis; Steroids
PubMed: 35384191
DOI: 10.1111/dth.15490 -
Clinical Rheumatology Oct 2021Pyoderma gangrenosum (PG) is a rare, rapidly progressive neutrophilic dermatosis commonly associated with systemic inflammatory diseases. We aimed to characterize the...
INTRODUCTION/OBJECTIVES
Pyoderma gangrenosum (PG) is a rare, rapidly progressive neutrophilic dermatosis commonly associated with systemic inflammatory diseases. We aimed to characterize the association of PG and inflammatory arthritis, as little is known outside of case reports and small cohort studies.
METHOD
We performed a systematic review in PubMed, EMBASE, and Scopus from inception to present using the terms arthritis and pyoderma gangrenosum. Patient demographics, clinical presentation, and treatment outcomes were recorded. Descriptive statistics and stratified analysis were used to compare factors of interest by type of arthritis.
RESULTS
A total of 1399 articles were screened, and 129 patients with inflammatory arthritis and PG were included in the review. The most common types of arthritis were rheumatoid arthritis (RA) (50.4%), inflammatory bowel disease (IBD)-associated arthritis (10.9%), and psoriatic arthritis (8.5%). In the vast majority of cases, joint symptoms preceded PG, by a median of 10 years (inter-quartile range [IQR] 5-16). Corticosteroid monotherapy and biologic therapies, used alone or in combination, resulted in improvement or complete resolution of ulcers 71.4% and 67.3% of the time, respectively. Within the latter, infliximab, adalimumab, and anakinra were most successful in inducing remission overall. RA and non-RA did not differ significantly in treatment success or healing time.
CONCLUSIONS
This study shows that PG is frequently preceded by inflammatory arthritis, most commonly RA. Clinicians used a wide variety of treatment regimens with variable outcomes. While larger studies are needed to standardize the treatment of inflammatory arthritis-associated PG, this study suggests that in addition to systemic corticosteroids, biologic medications can be effective treatment options for these patients.
KEY POINTS
• Inflammatory arthritis, most commonly rheumatoid arthritis, often precedes rather than follows pyoderma gangrenosum. • Other forms of arthritis associated with PG included IBD-associated arthritis and psoriatic arthritis. • Biologic therapies, such as infliximab, adalimumab, and anakinra, were largely successful in treating arthritis-associated pyoderma gangrenosum and may play an important role in corticosteroid-sparing therapy or in a maintenance regimen for this subset of patients. • The type of inflammatory arthritis associated with pyoderma gangrenosum may not be a helpful treatment guide as it was not significantly associated with treatment outcomes or healing time.
Topics: Adalimumab; Arthritis, Rheumatoid; Humans; Inflammatory Bowel Diseases; Infliximab; Pyoderma Gangrenosum
PubMed: 34002351
DOI: 10.1007/s10067-021-05768-7 -
Giornale Italiano Di Dermatologia E... Oct 2014Pyoderma gangrenosum (PG) is a rare, chronic neutrophilic dermatosis of unknown etiology. The world wide incidence is estimated to be around 3-10 cases per million... (Review)
Review
Pyoderma gangrenosum (PG) is a rare, chronic neutrophilic dermatosis of unknown etiology. The world wide incidence is estimated to be around 3-10 cases per million population per year. In 50-70% of cases inflammatory bowel diseases, hematological malignancies or rheumatologic disorders are associated to PG. Although the etiology is uncertain, the dysregulation of the immune system appears to be implied. Pathergy is the most important triggering factor of PG. Indeed, 20-30% of patients report the onset of PG following trivial trauma. Four main variants of PG have been described, namely classic, pustular, bullous, and vegetative forms. The classic form of PG is characterized by ulcers with a raised, undermined, inflammatory border. Intense pain is generally associated to PG. The diagnosis is mainly clinical and of exclusion. The differential diagnosis should take into account infections, vascular disorders and malignancies. The clinical course can be explosive and rapidly progressive or indolent and gradually progressive. Often patients develop only one episode and the overall prognosis is good but extremely influenced by the underlying disorders. Local therapy, mainly with topic steroids is used for mild to moderate lesions. For severe forms of PG a systemic therapy with glucocorticoids and/or other drugs such as tacrolimus, cyclosporine, etc. is needed. This paper is a systematic review of literature on PG.
Topics: Anti-Inflammatory Agents; Arthritis; Dermatologic Agents; Diagnosis, Differential; Disease Progression; Disease Susceptibility; Female; Humans; Immunologic Factors; Immunosuppressive Agents; Inflammatory Bowel Diseases; Leukapheresis; Lymphoproliferative Disorders; Male; Pregnancy; Pregnancy Complications; Prognosis; Pyoderma Gangrenosum; Skin Ulcer; Thalidomide
PubMed: 25213386
DOI: No ID Found -
Experimental Dermatology Apr 2022Pyoderma gangrenosum is a painful recurrent ulcerative neutrophilic dermatosis in which the pathogenesis is incompletely defined. Current evidence suggests that PG is... (Review)
Review
Pyoderma gangrenosum is a painful recurrent ulcerative neutrophilic dermatosis in which the pathogenesis is incompletely defined. Current evidence suggests that PG is associated with dysregulation of components of both the innate and adaptive immune system with dysregulation of neutrophil function and contribution of the Th17 immune axis. PG can be present in numerous heterogeneous clinical presentations and be associated with multiple inflammatory conditions including rheumatoid arthritis, inflammatory bowel disease and hidradenitis suppurativa. However, no critical evaluation of the observed molecular characteristics in PG studies in association with their clinical findings has been assessed. Additionally, emerging evidence suggests a potential role for other cell types and immune pathways including B cells, macrophages, autoantibodies and the complement system in PG, although these have not yet been integrated into the pathogenesis of disease. This systematic review aims to critically evaluate the current molecular observations regarding the pathogenesis of PG and discuss associations with clinical characteristics as well as the evidence supporting novel cell types and immune pathways in PG.
Topics: Dermatitis; Hidradenitis Suppurativa; Humans; Inflammatory Bowel Diseases; Neutrophils; Pyoderma Gangrenosum
PubMed: 35114021
DOI: 10.1111/exd.14534 -
American Journal of Clinical Dermatology Aug 2018There is little consensus regarding the prevalence and distribution of underlying systemic diseases among patients with pyoderma gangrenosum. (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
There is little consensus regarding the prevalence and distribution of underlying systemic diseases among patients with pyoderma gangrenosum.
OBJECTIVE
The objective of this study was to synthesize existing data on the prevalence of associated systemic diseases in patients with pyoderma gangrenosum.
METHODS
We performed a systematic review and meta-analysis of observational studies in MEDLINE, EMBASE, and Scopus (1823-2017). The quality of evidence was assessed using a modified Newcastle-Ottawa Scale. A meta-analysis was performed using random-effects models to estimate pooled prevalence rates with 95% confidence intervals.
RESULTS
Twenty-one eligible studies comprising 2611 patients with pyoderma gangrenosum were included in the quantitative synthesis. The overall random-effects pooled prevalence of associated systemic diseases was 56.8% (95% confidence interval 45.5-67.4). The leading underlying disease was inflammatory bowel disease (17.6%; 95% confidence interval 13.0-22.7), followed by arthritis (12.8%; 95% confidence interval 9.2-16.9), hematological malignancies (8.9%; 95% confidence interval 6.5-11.6), and solid malignancies (7.4%; 95% confidence interval 5.8-9.1). In 16.3% (95% confidence interval 7.7-27.1) of cases, the onset of pyoderma gangrenosum was attributed to the pathergy phenomenon.
CONCLUSIONS
More than half of patients with pyoderma gangrenosum present with a relevant underlying disease. Inflammatory bowel disease and arthritis are the most frequently associated diseases. Relative to the reported literature, the pooled prevalence of arthritis and hematological malignancies is lower, while the pooled prevalence of solid malignancies is higher. Owing to the high level of heterogeneity among most of the comparisons, results should be interpreted with caution.
Topics: Arthritis; Humans; Inflammatory Bowel Diseases; Neoplasms; Pyoderma Gangrenosum
PubMed: 29721816
DOI: 10.1007/s40257-018-0356-7 -
The British Journal of Dermatology Jun 2015Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful skin ulcerations for which treatment can be challenging. The genetic basis of PG may... (Review)
Review
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful skin ulcerations for which treatment can be challenging. The genetic basis of PG may provide a better understanding of the disease and new targets for treatment. We systematically reviewed the published literature regarding the syndromes and genetic mutations associated with PG. A literature search was performed through the clinical queries PubMed (National Library of Medicine) database and the Cochrane database. The studies were assessed and then categorized as relating to syndromes or specific gene mutations. Two hundred and eight articles were identified, describing 823 cases of PG. A total of 537 (65·2%) cases were associated with inflammatory bowel disease, 133 (16·1%) with polyarthritis and 103 (12·5%) with haematological disorders. Thirty-one cases of pyogenic arthritis, pyoderma gangrenosum and acne, and its variants, were identified. Two patients had mutations in MTHFR and two had mutations in JAK2. Fourteen (1·7%) cases were familial. PG responded to different treatments depending on the setting. For example, treatment with B vitamins improved PG in cases of mutations in MTHFR, whereas patients with myelodysplastic syndrome improved with thalidomide treatment. PG can occur in isolation, associated with systemic disease or as part of various syndromes. Different genetic causes may be best treated with particular treatments. Understanding its genetic basis can help elucidate new potential targets for drug development.
Topics: Acne Vulgaris; Arthritis; Child; Child, Preschool; Forecasting; Hematologic Diseases; Humans; Irritable Bowel Syndrome; Janus Kinase 2; Methylenetetrahydrofolate Reductase (NADPH2); Mutation; Pyoderma Gangrenosum
PubMed: 25350484
DOI: 10.1111/bjd.13493