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Journal of Wound Care Oct 2022Pyoderma gangrenosum (PG) is an often-misdiagnosed, painful, inflammatory and ulcerative skin disorder. It is an orphan disease, where standard wound treatments such as...
OBJECTIVE
Pyoderma gangrenosum (PG) is an often-misdiagnosed, painful, inflammatory and ulcerative skin disorder. It is an orphan disease, where standard wound treatments such as sharp surgical debridement are contraindicated. This retrospective case series sought to evaluate the application of dehydrated human amnion/chorion membrane (DHACM) as a skin substitute in cases that were refractory to a range of standard-of-care techniques.
METHOD
This retrospective case series involved wounds which failed to close with standard escalating treatments, including anti-inflammatory and immunosuppressive therapies. Subjects were transitioned to DHACM and wound sizes were monitored until closure.
RESULTS
Wounds (n=5) for all three subjects had stalled with standard therapies for at least 2.5 months but responded quickly to routinely applied DHACM treatments, and closure was achieved in each case.
CONCLUSION
This retrospective pilot case series examined the use of DHACM as an alternative wound treatment for PG patients failing standard therapies. DHACM treatments re-initiated the trajectory towards wound closure for each stalled PG ulcer. The results suggest a treatment algorithm starting with early recognition, wound closure via treatment escalation, and lastly a gradual reduction in treatment for durable closure. DHACM treatment should be formally evaluated as an adjunct to PG ulcers that have remained refractory to more commonly used immunomodulating therapies.
Topics: Amnion; Chorion; Humans; Pyoderma Gangrenosum; Rare Diseases; Retrospective Studies; Treatment Outcome; Wound Healing
PubMed: 36240792
DOI: 10.12968/jowc.2022.31.10.808 -
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 -
Romanian Journal of Morphology and... 2015Cutaneous symptoms are characteristic for the autoinflammatory disorders (AIDs), both in the classical autoinflammatory phenotype and in most disorders included in this... (Review)
Review
Cutaneous symptoms are characteristic for the autoinflammatory disorders (AIDs), both in the classical autoinflammatory phenotype and in most disorders included in this syndrome, but they are not specific and inconstant. Several skin disorders (pyoderma gangrenosum and pustular acne) may be encountered either isolate or associated with autoinflammatory symptoms, forming well-defined clinical entities within the autoinflammatory syndrome. The high prevalence of cutaneous manifestations is an important characteristic of AIDs. The presence of cutaneous symptoms in AIDs opens the perspective of understanding the contribution of innate immunity mechanisms involved in skin pathology. It is possible that many diseases present the alteration, in various degrees, of the innate immune mechanisms. Recently, dermatology faced two challenges connected to AIDs. The first involves the diagnosis of skin symptoms in a clinical autoinflammatory setting and the investigative approach to identify a disorder classified as AID. The second is to identify the altered mechanisms of inborn immunity among the pathogenetic mechanisms of known dermatological diseases (e.g., neutrophilic dermatoses). On the other hand, cutaneous symptoms are in certain cases regarded as a criterion to asses the efficacy of specific or non-specific therapies with monoclonal antibodies in disorders included in AIDs. Dermatology mostly benefits from the identification and knowledge of AIDs due to the role of innate immunity in skin pathogeny and also due to the large extent of clinical forms resulting from the association of skin symptoms with other disorders included in this group.
Topics: Acne Vulgaris; Anti-Inflammatory Agents, Non-Steroidal; Antibodies, Monoclonal; Complement System Proteins; Dermatology; Gene Expression Regulation; Humans; Immunity, Innate; Inflammation; Interleukin-1beta; Macrophages; Mutation; Phenotype; Pyoderma Gangrenosum; Recombinant Proteins; Skin; Skin Diseases; Skin Physiological Phenomena
PubMed: 25826481
DOI: No ID Found -
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 -
Postepy Higieny I Medycyny... Mar 2016Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG). The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment.
METHODS
Systematic review of the literature in PubMed using the search terms "Wegener granulomatosis AND Pyoderma gangrenosum" and case report.
RESULTS
The finding of 22 reports in the literature (PubMed) suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide.
DISCUSSION
The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.
Topics: Face; Female; Granulomatosis with Polyangiitis; Humans; Neck; Necrosis; Neutrophils; Pyoderma Gangrenosum; Skin Ulcer; Young Adult
PubMed: 27117096
DOI: 10.5604/17322693.1197372 -
The Medical Journal of Malaysia Aug 2007Pyoderma Gangrenosum (PG) is a non-infective, necrotising neutrophilic dermatosis. Many diseases may mimic PG. It may be idiopathic or may be associated with a systemic...
Pyoderma Gangrenosum (PG) is a non-infective, necrotising neutrophilic dermatosis. Many diseases may mimic PG. It may be idiopathic or may be associated with a systemic disorder. We report a case of PG in association with ulcerative colitis.
Topics: Aged; Colitis, Ulcerative; Humans; Malaysia; Male; Outcome Assessment, Health Care; Pyoderma Gangrenosum
PubMed: 18246920
DOI: No ID Found -
Orthopaedic Surgery Jan 2023Pyoderma gangrenosum (PG) is a reactive, noninfectious, neutrophilic dermatosis. Diagnosis of PG is based on exclusion, due to lack of availability of a confirmatory... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a reactive, noninfectious, neutrophilic dermatosis. Diagnosis of PG is based on exclusion, due to lack of availability of a confirmatory test. PG is not caused by infection or gangrene. Misdiagnosis or delayed diagnosis of PG can lead to devastating results.
CASE PRESENTATION
In this report, we present a patient with a delayed diagnosis of PG lesion on right hand. Despite initial surgical treatment, the wound was aggravated, and amputation was considered; however, it was eventually treated successfully with an autologous split thickness skin graft.
CONCLUSIONS
Knowledge of the PG is essential to actively consider PG in early stage to help facilitate immediate treatment and avoid unnecessary interventions that may worsen the outcome.
Topics: Humans; Pyoderma Gangrenosum; Hand
PubMed: 36222208
DOI: 10.1111/os.13538 -
Dermatology Online Journal Oct 2011A 79-year-old woman was admitted to our hospital with pustular pyoderma gangrenosum and an associated IgG kappa monoclonal gammopathy. The patient is currently being...
A 79-year-old woman was admitted to our hospital with pustular pyoderma gangrenosum and an associated IgG kappa monoclonal gammopathy. The patient is currently being evaluated for possible multiple myeloma. IgG multiple myeloma and IgG monoclonal gammopathies are very rare in patients with pyoderma gangrenosum. The skin lesions are improving with the use of prednisone.
Topics: Aged; Anti-Bacterial Agents; Anti-Inflammatory Agents; Combined Modality Therapy; Debridement; False Positive Reactions; Female; Fluorescent Treponemal Antibody-Absorption Test; Humans; Immunoglobulin G; Leg Ulcer; Multiple Myeloma; Neutrophils; Paraproteinemias; Prednisone; Pyoderma Gangrenosum; Reoperation
PubMed: 22031643
DOI: No ID Found -
Polskie Archiwum Medycyny Wewnetrznej Sep 2016
Topics: Adenine; Cyclosporine; Drug Therapy, Combination; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Male; Middle Aged; Piperidines; Prednisone; Pyoderma Gangrenosum; Pyrazoles; Pyrimidines
PubMed: 27698335
DOI: 10.20452/pamw.3578 -
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