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Frontiers in Medicine 2023Pyoderma gangrenosum (PG) is a rare autoinflammatory ulcerative neutrophilic skin disease. Its clinical presentation is a rapidly progressing painful skin ulcer with... (Review)
Review
Pyoderma gangrenosum (PG) is a rare autoinflammatory ulcerative neutrophilic skin disease. Its clinical presentation is a rapidly progressing painful skin ulcer with ill-defined borders and surrounding erythema. The pathogenesis of PG is complex and not fully understood. Clinically, patients with PG often have various systemic diseases, the most common being inflammatory bowel disease (IBD) and arthritis. Due to the lack of specific biological markers, diagnosing PG remains difficult, which easily resulting in misdiagnosis. Some validated diagnostic criteria have been applied in clinical practice that facilitate its diagnosis. The treatment of PG currently consists mainly of immunosuppressive and immunomodulatory agents, especially biological agents, which have bright prospects for PG therapy. After the systemic inflammatory response is controlled, the problem of wounds becomes the main contradiction in PG treatment. Surgery is not controversial for PG, increasing evidence shows that with adequate systemic treatment, the benefits of reconstructive surgery for patients are increasing.
PubMed: 36865058
DOI: 10.3389/fmed.2023.1134939 -
European Journal of Case Reports in... 2021Superficial granulomatous pyoderma is a relatively rare variant of pyoderma gangrenosum, characterized by superficial ulceration with a vegetative margin and a clean...
UNLABELLED
Superficial granulomatous pyoderma is a relatively rare variant of pyoderma gangrenosum, characterized by superficial ulceration with a vegetative margin and a clean granulating base. Ulcers in superficial granulomatous pyoderma are typically located on the trunk and may follow minor trauma. It may be misdiagnosed as classic pyoderma gangrenosum, despite having distinct characteristics. Here we report a case of superficial granulomatous pyoderma successfully treated with intravenous immunoglobulin (IVIg).
LEARNING POINTS
Superficial granulomatous pyoderma is a rare variant of pyoderma gangrenosum.Histopathological and clinical characteristics of superficial granulomatous pyoderma are distinct from those of classic pyoderma gangrenosum.Superficial granulomatous pyoderma is often slow-growing and is only rarely linked to underlying systemic disease.IVIg may be an effective treatment option for superficial granulomatous pyoderma.
PubMed: 34671571
DOI: 10.12890/2021_002656 -
Clinical, Cosmetic and Investigational... 2023Pyoderma gangrenosum (PG) is a rare, autoinflammatory disease leading to aseptic ulcers which carries a significant disease burden and is often difficult to treat, with... (Review)
Review
Pyoderma gangrenosum (PG) is a rare, autoinflammatory disease leading to aseptic ulcers which carries a significant disease burden and is often difficult to treat, with many patients failing first-line treatment and requiring additional therapies. Such cases are typically referred to in the literature as "recalcitrant", "refractory", or "resistant", though little is known about the clinical characteristics of such cases. We performed a narrative literature review to characterize patient demographics and clinical course associated with difficult to treat pyoderma gangrenosum cases in order to identify trends to guide future clinical management and therapeutic innovation. We identified 148 cases with clinical manifestations and associated patient demographics stratified by ulcer and patient features. Consistent with previous work, a greater prevalence of PG was observed among female patients and those with a history of inflammatory bowel disease, however interestingly despite an aggressive course to their PG, few patients had comorbidities complicating their disease course. Additionally, despite the requirement of three or more treatments for most patients' disease to resolve, the majority healed within the typical window observed in previous clinical studies with low rates of recurrence. Biologics were the most common medication patients were on at time of remission. Collectively, our results suggest a potential benefit for a reduced threshold for biologic initiation in PG patients and a need for standardization of language in the field to facilitate treatment outcomes comparisons and interventions.
PubMed: 37581011
DOI: 10.2147/CCID.S381490 -
Anais Brasileiros de Dermatologia 2020The severe bacterial diseases discussed herein are those that present dermatological lesions as their initial manifestations, for which the dermatologist is often called...
The severe bacterial diseases discussed herein are those that present dermatological lesions as their initial manifestations, for which the dermatologist is often called upon to give an opinion or is even the first to examine the patient. This review focuses on those that evolve with skin necrosis during their natural history, that is, necrotizing fasciitis, Fournier gangrene, and ecthyma gangrenosum. Notice that the more descriptive terminology was adopted; each disease was individualized, rather than being referred by the generic term "necrotizing soft tissue infections". Due to their relevance and increasing frequency, infections by methicillin-resistant Staphylococcus aureus (MRSA) were also included, more specifically abscesses, furuncle, and carbuncle, and their potential etiologies by MRSA. This article focuses on the epidemiology, clinical dermatological manifestations, methods of diagnosis, and treatment of each of the diseases mentioned.
Topics: Anti-Bacterial Agents; Ecthyma; Fasciitis, Necrotizing; Humans; Methicillin-Resistant Staphylococcus aureus; Skin Diseases, Bacterial; Soft Tissue Infections; Staphylococcal Infections
PubMed: 32507327
DOI: 10.1016/j.abd.2020.04.003 -
International Wound Journal Oct 2020Pyoderma gangrenosum (PG) is a rare disease of unknown aetiology, first described over a century ago. Initially thought to have an infectious cause, and now primarily... (Review)
Review
Pyoderma gangrenosum (PG) is a rare disease of unknown aetiology, first described over a century ago. Initially thought to have an infectious cause, and now primarily considered an autoinflammatory condition, PG continues to be poorly understood, commonly misdiagnosed, and difficult to treat. In this review, we discuss the journey of our understanding of PG to date, including first descriptions, challenges with diagnosis, presumed pathogenesis, and treatments used. We highlight major historical landmarks and their importance, explain the rationale behind current investigations, note outstanding gaps in knowledge, and explore the future directions of PG research. We summarise what we have known, what we are working on knowing, and what we have yet to explore about PG, illustrating overall trends to invigorate future research.
Topics: Humans; Pyoderma Gangrenosum
PubMed: 32378319
DOI: 10.1111/iwj.13389 -
Arthritis & Rheumatology (Hoboken, N.J.) Feb 2022Dominantly inherited PSTPIP1 mutations cause a spectrum of autoinflammatory manifestations epitomized by PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum,...
OBJECTIVE
Dominantly inherited PSTPIP1 mutations cause a spectrum of autoinflammatory manifestations epitomized by PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome.). The connections between PSTPIP1 and PAPA syndrome are poorly understood, although evidence suggests involvement of pyrin inflammasome activation. Interleukin-18 (IL-18) is an inflammasome-activated cytokine associated with susceptibility to macrophage activation syndrome (MAS). This study was undertaken to investigate an association of IL-18 with PAPA syndrome.
METHODS
Clinical and genetic data and serum samples were obtained from patients referred to institutions due to symptoms indicative of PAPA syndrome. Serum IL-18, IL-18 binding protein (IL-18BP), and CXCL9 levels were assessed by bead-based assay, and free IL-18 levels were assessed by enzyme-linked immunosorbent assay.
RESULTS
The symptoms of PSTPIP1-positive patients with PAPA syndrome overlapped with those of mutation-negative patients with PAPA-like conditions, but mutation-positive patients had earlier onset and a greater proportion had a history of arthritis. We found uniform elevation of total serum IL-18 in treated PAPA syndrome patients at levels nearly as high as those seen in NLRC4-associated autoinflammation with infantile enterocolitis patients, and well above levels found in most familial Mediterranean fever patients. Serum IL-18 elevation in PAPA syndrome patients persisted despite fluctuations in disease activity. Levels of the soluble IL-18 antagonist IL-18BP were modestly elevated, and PAPA syndrome patients had detectable free IL-18. PAPA syndrome was rarely associated with elevation of CXCL9, an indicator of interferon-γ activity, but no PAPA syndrome patients had a history of MAS.
CONCLUSION
PAPA syndrome is a refractory and often disabling monogenic autoinflammatory disease associated with chronic and unopposed elevation of serum IL-18 levels but not with risk of MAS. These findings affect our understanding of the diseases in which IL-18 is overproduced and suggest a link between pyrin inflammasome activation, IL-18, and autoinflammation, without susceptibility to MAS.
Topics: Acne Vulgaris; Adaptor Proteins, Signal Transducing; Adolescent; Adult; Arthritis, Infectious; Child; Child, Preschool; Cytoskeletal Proteins; Female; Humans; Infant; Interleukin-18; Male; Middle Aged; Mutation; Pyoderma Gangrenosum; Retrospective Studies; Young Adult
PubMed: 34492165
DOI: 10.1002/art.41976 -
Indian Journal of Dermatology,... 2023Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and... (Review)
Review
BACKGROUND
Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and prognosis of pyoderma gangrenosum.
AIMS
We aimed to document clinicopathologic and prognostic data of the patients with pyoderma gangrenosum.
METHODS
In this retrospective observational study, we reviewed case records of patients diagnosed with pyoderma gangrenosum between 1999-2019.
RESULTS
Fifty-three patients were identified by reviewing medical records for skin biopsy; of these, 37 were men and 16 were women. Mean age at onset was 43.3 ± 18.5 years. The most frequently affected area was the lower extremities (60.4%), followed by the head and neck (17.0%). The most common subtype was ulcerative (47.2%), followed by bullous (22.6%). 30 cases had underlying diseases and the most common were malignancy (24.5%), followed by inflammatory bowel diseases (18.9%). The proportion of cases with history of trauma were significantly higher in post-operative type (100%) as compared to the bullous type (8.3%). Histologic features of granulation tissue were frequently found in post-operative type (66.7%) and bullous type (58.3%). Granulomas were predominantly found in bullous type (58.3%). Age <60 years appeared to be significantly associated with multiple lesions. Partial-to-complete remission was observed in 40 cases (75.5%). Nine (17.0%) cases experienced recurrence with a median progression-free period of six months (interquartile range of 3.0-9.0 months). Cases with underlying hematologic disorders and the bullous subtype were significantly associated with early recurrence.
LIMITATIONS
This study was a single-centre study with a retrospective design.
CONCLUSION
Pyoderma gangrenosum appears to have ethnic differences. Underlying haematologic disorders and bullous subtype have a worse prognosis. However, the type of histopathology did not correlate with the clinical outcome of pyoderma gangrenosum.
Topics: Male; Humans; Female; Young Adult; Adult; Middle Aged; Pyoderma Gangrenosum; Retrospective Studies; Inflammatory Bowel Diseases; Prognosis; Republic of Korea; Observational Studies as Topic
PubMed: 35389018
DOI: 10.25259/IJDVL_968_20 -
Plastic and Reconstructive Surgery.... Jan 2024Pyoderma gangrenosum is a neutrophilic dermatosis characterized by immune dysfunction and pathergy. Thus, it is frequently seen in patients with underlying systemic... (Review)
Review
Pyoderma gangrenosum is a neutrophilic dermatosis characterized by immune dysfunction and pathergy. Thus, it is frequently seen in patients with underlying systemic illnesses or postoperatively. For the performance of the debridement or closure of the resultant defect, plastic surgeons are often involved in the care of pyoderma patients. However, both procedures may exacerbate the injury. Therefore, plastic surgeons must be familiar with the presentation of postsurgical pyoderma to avoid further damage and safely repair related soft tissue defects. A systematic search of the PubMed/Medline database was performed using the following keywords: "pyoderma gangrenosum" and "surgery." This online database search has identified 656 studies published between 1958 and 2022. Only reconstructed cases of postsurgical pyoderma gangrenosum were selected. Twenty-eight patients who developed pyoderma after dermatologic, plastic, orthopedic, cardiovascular, general, or obstetric surgery were included in this study. The average time to the PG presentation and diagnosis was 5.5 and 17 days, respectively. Diagnostic scoring tools were not used, and the diagnosis was primarily based on histopathology after repeated treatment failures. The patients received split- or full-thickness skin grafts, local, pedicled, and free flaps. An estimated 82.1% underwent skin grafting, whereas 42.9% underwent flap reconstruction. In addition, 21.4% got both the graft and flap. Accurate diagnosis of PSPG, prevention of further surgical injury, and timely medical management are vital for improving patient outcomes. Reconstruction can be performed, if required. However, despite the availability of different reconstructive techniques, there is no standard approach to the management of the PSPG.
PubMed: 38250211
DOI: 10.1097/GOX.0000000000005505 -
JPMA. the Journal of the Pakistan... Jan 2022Pyoderma Gangrenosum (PG) is a rare, debilitating, and painful disease of the skin. Its aetiology and pathophysiology are not well understood. However, it is known that... (Review)
Review
Pyoderma Gangrenosum (PG) is a rare, debilitating, and painful disease of the skin. Its aetiology and pathophysiology are not well understood. However, it is known that PG is not bacterial in origin, as previously believed. A significant number of cases of PG report a phenomenon called pathergy, which is characterized by the appearance of new lesions after the application of trauma to the skin. This represents a unique challenge for surgeons in cases that are refractory to medical therapy. The objective of this study is to review past literature and report a case of PG in a 19-year old woman, who presented with recurrence, after undergoing skin grafting one year back. The patient was referred for split thickness skin grafting (STSG) to reduce the psychological and physical morbidity as a result of this disease.
Topics: Adult; Female; Humans; Pyoderma Gangrenosum; Skin; Skin Transplantation; Young Adult
PubMed: 35099456
DOI: 10.47391/JPMA.11-1109 -
International Journal of Molecular... Feb 2024Pyoderma gangrenosum (PG) is an uncommon inflammatory dermatological disorder characterized by painful ulcers that quickly spread peripherally. The pathophysiology of PG... (Review)
Review
Pyoderma gangrenosum (PG) is an uncommon inflammatory dermatological disorder characterized by painful ulcers that quickly spread peripherally. The pathophysiology of PG is not fully understood; however, it is most commonly considered a disease in the spectrum of neutrophilic dermatoses. The treatment of PG remains challenging due to the lack of generally accepted therapeutic guidelines. Existing therapeutic methods focus on limiting inflammation through the use of immunosuppressive and immunomodulatory therapies. Recently, several reports have indicated the successful use of biologic drugs and small molecules administered for coexisting diseases, resulting in ulcer healing. In this review, we summarize the discoveries regarding the pathophysiology of PG and present treatment options to raise awareness and improve the management of this rare entity.
Topics: Humans; Pyoderma Gangrenosum; Immunosuppressive Agents; Inflammation; Biological Products; Immunomodulation
PubMed: 38397117
DOI: 10.3390/ijms25042440