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Therapeutic Apheresis and Dialysis :... Apr 2022Pyoderma gangrenosum (PG), a chronic aseptic inflammatory skin disease characterized by skin ulcers with elevated and undermined borders, is resistant to conventional...
Pyoderma gangrenosum (PG), a chronic aseptic inflammatory skin disease characterized by skin ulcers with elevated and undermined borders, is resistant to conventional therapies. PG is elicited by activated neutrophils and macrophages and is often associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis, aortitis syndrome, and hematopoietic disorders. This single-center study assessed the efficacy and safety of selectively depleting myeloid-lineage leukocytes in patients with PG. Patients with PG, aged 20 or over, received 5 or 10 treatment sessions of granulocyte and monocyte adsorption apheresis (GMA), once or twice a week. Treatment efficacy was assessed based on the rate of skin ulcer reduction, the visual analog scale of pain, and the physician's global assessment of the skin lesions. A complete response (CR) was obtained in eight patients, a nearly complete response (nCR) in three patients, and a partial response (PR) in two patients. In four of the other six, the disease remained stable (SD) and in two we observed disease progression (PD). No severe adverse events were recorded. Our results suggest that GMA is a useful and safe treatment modality for PG.
Topics: Adsorption; Adult; Blood Component Removal; Colitis, Ulcerative; Granulocytes; Humans; Monocytes; Pyoderma Gangrenosum; Treatment Outcome; Young Adult
PubMed: 34369050
DOI: 10.1111/1744-9987.13720 -
International Wound Journal Oct 2019Peristomal pyoderma gangrenosum (PPG) is an under-recognised and difficult condition to treat. We describe a case series using a novel topical combination therapy that... (Review)
Review
Peristomal pyoderma gangrenosum (PPG) is an under-recognised and difficult condition to treat. We describe a case series using a novel topical combination therapy that promotes wound healing and allows for adhesion of the stoma appliance. A crushed oral prednisolone tablet mixed with Stomahesive Protective Powder (ConvaTec) was applied topically to seven patients with PPG and resulted in pain relief and wound healing in six of seven patients. Only one patient experienced recurrence. The novel topical therapy we describe is cost-effective, readily available, and easily applied in any inpatient or outpatient setting.
Topics: Administration, Topical; Aged; Female; Follow-Up Studies; Humans; Male; Middle Aged; Prednisolone; Pyoderma Gangrenosum; Retrospective Studies; Risk Assessment; Sampling Studies; Surgical Stomas; Talc; Wound Healing
PubMed: 31298491
DOI: 10.1111/iwj.13164 -
Wound Repair and Regeneration :... May 2022The objective of this study was to develop a pilot physician driven patient pyoderma gangrenosum (PG) registry to summarise patient baseline demographics, PG-related...
The objective of this study was to develop a pilot physician driven patient pyoderma gangrenosum (PG) registry to summarise patient baseline demographics, PG-related medical history, treatments, and outcomes for patients with pyoderma gangrenosum. Standardised patient information was collected prospectively during clinical encounters between December 2019 and July 2021 at a single academic institution. Eligibility criteria for the study was a diagnosis of pyoderma gangrenosum determined by a PARACELSUS score of at least 10 for ulcerative patients. Main outcome measures included demographic data, PG related history and comorbidities, past and current treatments, healing outcomes, hospitalisations and recurrences of PG. The Pyoderma Gangrenosum Study (PYGAS) Registry currently includes 52 patients with 56 target lesions of four distinct PG subtypes (41 ulcerative, 12 peristomal, 2 vegetative and 1 bullous). For the 38 patients with 41 total ulcerative PG lesions, referrals to our institution most commonly came from dermatologists (42.1%). The median follow-up time in our initial registry was 5.5 months (95% CI = 4.1-11.5 months), with average time between follow-up visits at 1.1 months. These ulcers were most commonly treated with first-line systemic immunosuppressants (70.6%), such as corticosteroids or cyclosporine. Additional use of systemic immunomodulators at baseline visit was statistically significantly associated with healing (P = 0.048). This pilot study suggests that use of systemic immunomodulators has an impact on healing of PG patients. Wound care regimens are variable, and assessing their impact on treatment outcomes could be challenging. Standardisation of both wound care regimens and data collection in prospective clinical studies is necessary to assess their impact in PG treatment outcomes.
Topics: Humans; Pilot Projects; Prospective Studies; Pyoderma Gangrenosum; Registries; Wound Healing
PubMed: 35363927
DOI: 10.1111/wrr.13005 -
European Review For Medical and... Jul 2022Pyoderma Gangrenosum (PG) is an immune-mediated neutrophilic dermatosis, characterized by large painful ulcers occurring in various body segments. It can be associated... (Review)
Review
OBJECTIVE
Pyoderma Gangrenosum (PG) is an immune-mediated neutrophilic dermatosis, characterized by large painful ulcers occurring in various body segments. It can be associated to Inflammatory Bowel Disease (IBD) including both Ulcerative Colitis and Crohn Disease. Prompt and effective management is fundamental, due to its high morbidity and mortality rates. By presenting our clinical experience, we aimed at showing the efficacy of a combined therapeutic approach, in which the best of every specialty cooperates managing this hazardous disease.
PATIENTS AND METHODS
We report on two patients attending our outpatient clinic with ulcerative skin lesions at the level of the back. Patient 1 suffered from Crohn disease and Patient 2 presented a positive history of abdominal pain, diarrhea with mucus and blood in the stool. Histological exam was performed with final diagnosis of PG associated with IBD. A Literature review was carried out in order to highlight the role of combined clinical-surgical management of PG in adult patients with IBD.
RESULTS
Complete resolution of the lesions was achieved in 4 months and 3 months for each patient respectively without relapse. PubMed was searched from 2000 to 2020 with the following keywords: (Pyoderma) AND/OR (Pyoderma Gangrenosum) AND (Inflammatory Bowel Disease) AND/OR (Ulcerative Colitis) AND/OR (Crohn Disease) AND (Management). Seven papers were included (4 case reports, 2 case series, 1 comprehensive review) and reviewed using a descriptive checklist.
CONCLUSIONS
PG should be treated by dedicated multidisciplinary teams, in which every specialist plays a crucial role from the diagnosis to the treatment and up to the long-term follow-up.
Topics: Adult; Chronic Disease; Colitis, Ulcerative; Crohn Disease; Humans; Inflammatory Bowel Diseases; Pyoderma Gangrenosum; Recurrence
PubMed: 35916817
DOI: 10.26355/eurrev_202207_29308 -
Journal Der Deutschen Dermatologischen... May 2022Pyoderma gangrenosum is an ulcerative autoinflammatory disease, lacking distinct histopathological characteristics to differentiate from other ulcerating conditions,...
BACKGROUND AND OBJECTIVES
Pyoderma gangrenosum is an ulcerative autoinflammatory disease, lacking distinct histopathological characteristics to differentiate from other ulcerating conditions, like venous leg ulcers. The objective of this study was therefore to find histopathological characteristics of pyoderma gangrenosum in a head-to-head comparison to venous leg ulcers.
PATIENTS AND METHODS
Eight tissue samples of pyoderma gangrenosum, twelve samples of venous leg ulcers and six samples of healthy skin were stained using an immunohistological multi antigen staining technology. The immune infiltrate and its spatial distribution were analyzed with contextual tissue cytometry software using fluorescence images.
RESULTS
The dense epidermal presence of CD45RO memory T cells and the rarefication of CD1a Langerhans cells in the epidermis were defining markers for pyoderma gangrenosum, implicating an epidermal immune reaction. In addition, high numbers of CD11c CD68 pro-inflammatory M1 macrophages were detected in the dermis, significantly extending the numbers seen in venous leg ulcers.
CONCLUSIONS
The histopathological differences found between pyoderma gangrenosum and venous leg ulcer can be used to distinguish between the two diseases and thus provide an important aid for the rapid initiation of adequate therapy. In addition, our data hint at an antigen-driven process in the epidermis, possibly involving CD1a Langerhans cells.
Topics: Humans; Leg Ulcer; Pyoderma Gangrenosum; Skin; Varicose Ulcer
PubMed: 35487858
DOI: 10.1111/ddg.14708 -
Wound Repair and Regeneration :... Mar 2021Chronic painful ulcers caused by pyoderma gangrenosum (PG) and cutaneous vasculitis remain to be a therapeutic challenge. Skin grafts have been used with success in... (Review)
Review
Chronic painful ulcers caused by pyoderma gangrenosum (PG) and cutaneous vasculitis remain to be a therapeutic challenge. Skin grafts have been used with success in selected cases but are generally avoided due to the fear of pathergy. The aim of this study was to investigate the safety and efficacy of skin grafting in the treatment of primary vasculitic ulcer (PVU) and PG. MEDLINE, Embase, the Cochrane Library, Clinicaltrial.gov, and WHO International Clinical Trials Registry Platform (ICTRP) were searched from inception to March 2020. A search for grey literature was conducted in May 2020. We included studies assessing the efficacy and safety of skin grafting in the treatment of PG and PVU. Studies were only included if skin grafting was performed after establishment of PG or PVU diagnosis. A total of 721 articles was identified through the database search of which 92 were included in this study. Ten articles were identified by handsearching the reference list of included studies. Finally, 102 articles describing 212 wounds in 153 patients were included. Complete healing was found in 75.5% of the wounds. The average time to complete was 10.8 weeks (95% CI 6.1-15.6). The mean donor site healing time was 1.9 weeks (95% CI 0.52-3.20). Pathergy was reported in 8 (5.2%) patients. One patient had severe infection related to skin grafting. A statistically significant difference in the number of patients receiving preoperative (P = .0079) and postoperative (P = .002) immunosuppressive therapy was found between the groups with complete healing/reduction and no improvement/aggravation. This systematic review finds the current evidence on efficacy and safety of skin grafting in treatment of PG and PVU to be promising but limited to the size and lack of studies superior to case reports and case series. Future placebo-controlled trials are required to draw a stronger conclusion.
Topics: Humans; Pyoderma Gangrenosum; Skin Transplantation; Ulcer; Vasculitis; Wound Healing
PubMed: 33377584
DOI: 10.1111/wrr.12882 -
Advances in Skin & Wound Care Nov 2022To review the litigation literature related to patients with pyoderma gangrenosum (PG) to characterize the legal issues they face. (Review)
Review
OBJECTIVE
To review the litigation literature related to patients with pyoderma gangrenosum (PG) to characterize the legal issues they face.
DATA SOURCES
Data sources include law databases Casetext and Lexis Nexis.
STUDY SELECTION
All disability and medical liability claims directly involving patients with PG were included.
DATA EXTRACTION
Data extraction came directly from Casetext and Lexis Nexis. All cases extracted came from September 1965 to December 2020. This resulted in 32 cases, 18 of which were excluded because they did not mention the plaintiff having a diagnosis of PG (n = 14).
DATA SYNTHESIS
The review found a total of six medical liability cases in which adverse health outcomes were reported, including prolonged suffering (2/6 cases), unnecessary treatments or procedures (2/6), exacerbation of disease (1/6 cases), and permanent scarring (1/6 cases). Despite this, two plaintiffs won their case and only one of them received any monetary award. Similarly, of the eight disability claims in the review, four were ruled in the favor of the plaintiffs and only two resulted in immediate awarding of benefits. Half of medical liability cases occurred in correctional facilities after the denial of appropriate care.
CONCLUSIONS
The findings demonstrate a need for access to specialty care in incarcerated populations. Cases that occurred in a hospital setting also stress the importance of initiatives such as telemedicine to efficiently increase access to care in a cost-effective manner. Further, PG has been recognized as a severe impairment in disability claims despite patients being denied because they were deemed able to perform other work.
Topics: Humans; Databases, Factual; Disabled Persons; Liability, Legal; Malpractice; Pyoderma Gangrenosum
PubMed: 35895601
DOI: 10.1097/01.ASW.0000855052.83443.d8 -
Journal of the American Academy of... Aug 2020Pyoderma gangrenosum (PG) classically presents with an acute inflammatory stage, characterized by rapid evolution of painful ulcerations. The pathergy associated with...
Pyoderma gangrenosum (PG) classically presents with an acute inflammatory stage, characterized by rapid evolution of painful ulcerations. The pathergy associated with PG lesions complicates disease management. Although PG is commonly treated with immunosuppression, some patients have refractory noninflammatory ulcers. In this subpopulation, there are case reports of successful surgical treatment. However, there is no consensus on optimal perioperative treatment for patients with PG undergoing surgery of any kind, PG related or otherwise. Therefore, we conducted a comprehensive literature review describing perioperative management practices and risk factors that may predict response to surgical intervention. We identified 126 cases of surgical intervention in patients with active PG; among these, only 16.7% experienced postoperative disease progression. No perioperative treatments or clinical risk factors were identified as statistically significant predictors of disease recurrence. Although limited by case series design and publication bias, this study is a valuable means of hypothesis generation for this rare condition.
Topics: Dermatologic Surgical Procedures; Humans; Perioperative Care; Pyoderma Gangrenosum; Recurrence; Secondary Prevention; Treatment Outcome
PubMed: 31927079
DOI: 10.1016/j.jaad.2020.01.002 -
Archives of Dermatological Research Mar 2024Peristomal pyoderma gangrenosum is an uncommon subtype of pyoderma gangrenosum mainly affecting stoma sites of patients with inflammatory bowel disease. While surgical... (Review)
Review
Peristomal pyoderma gangrenosum is an uncommon subtype of pyoderma gangrenosum mainly affecting stoma sites of patients with inflammatory bowel disease. While surgical treatments are often used to assist healing, little is known about the relationship between surgical interventions and the rate of recurrence of peristomal pyoderma gangrenosum. The aim of this study was to identify patient and clinical factors associated with peristomal pyoderma gangrenosum recurrence following surgical intervention. A multi-institutional retrospective case series and literature review was conducted to evaluate patient characteristics and perioperative treatment. Patients of any age with peristomal pyoderma gangrenosum undergoing surgical operations related to their pyoderma gangrenosum or due to another comorbidity were included. Descriptive statistics were used to characterize demographic information. Associations were evaluated using Wilcoxon's rank-sum test for continuous variables and Fisher's exact test for categorical data. Thirty-seven cases were included, 78.3% of which had a history of inflammatory bowel disease. Overall, 13 (35.1%) cases experienced recurrence at 30 days. There was no significant association identified between patient demographics, stoma location, surgical intervention, or perioperative treatment with rate of recurrence at 30 days post-operation. While no clinical risk factors or treatments were associated with recurrence, our work underscores the importance of a multidisciplinary approach to this disease to address gastrointestinal, dermatologic, and surgical components of treatment.
Topics: Humans; Pyoderma Gangrenosum; Retrospective Studies; Inflammatory Bowel Diseases; Postoperative Period; Risk Factors
PubMed: 38446235
DOI: 10.1007/s00403-024-02826-3 -
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