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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 -
Case Reports in Plastic Surgery & Hand... 2024We present a case of a patient addressed to our plastic surgery department after a breast tumor resection and subsequent necrosis and ulceration of the right breast. A...
We present a case of a patient addressed to our plastic surgery department after a breast tumor resection and subsequent necrosis and ulceration of the right breast. A diagnosis of pyoderma gangrenosum was made based on the clinical criteria, early recognition resulted in complete recovery without further reconstructive surgery.
PubMed: 38250334
DOI: 10.1080/23320885.2024.2304620 -
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 -
Frontiers in Medicine 2023Cutaneous manifestations of hematologic malignancy represent both a clinical challenge for the treating physician and a pathophysiological model for advancing the... (Review)
Review
Cutaneous manifestations of hematologic malignancy represent both a clinical challenge for the treating physician and a pathophysiological model for advancing the knowledge on individual neoplasms. Indeed, a growing body of evidence supports the concept of recurrent molecular defects associating with specific clinical features, as best exemplified by VEXAS. Herein neutrophilic and eosinophilic dermatoses of potential interest for both hematologists and dermatologists will be reviewed, including subcorneal pustular dermatosis-type IgA pemphigus, neutrophilic eccrine hidradenitis, Sweet's syndrome as well as myelodysplasia cutis and VEXAS, pyoderma gangrenosum, eosinophilic annular erythema, eosinophilic dermatosis of hematological malignancy, Wells syndrome and cutaneous involvement in hypereosinophilic syndromes. Possible management approaches are discussed for each, emphasizing scenarios that require treatment of the underlying condition to achieve remission at the skin level.
PubMed: 38249974
DOI: 10.3389/fmed.2023.1324258 -
Clinical, Cosmetic and Investigational... 2024Necrotizing fasciitis is a rare, severe, rapidly progressing disease with a high mortality rate. We report a case of a 72-year female with erythematous pemphigus who...
Necrotizing fasciitis is a rare, severe, rapidly progressing disease with a high mortality rate. We report a case of a 72-year female with erythematous pemphigus who developed erythema, swelling and ulceration on right vulva, groin, and thigh. The early clinical manifestations of the patient were nonspecific and easily misdiagnosed as cellulitis. However, upon the occurrence of ulceration and necrosis, deep fungal infection, pyoderma gangrenosum or lymphoproliferative disorders were considered. The pathology suggested IgG4-related diseases, plasmacytoma et al. But at last, surgical exploration and postoperative pathology confirmed the diagnosis of necrotizing fasciitis. The patient recovered after multiple aggressive surgical debridement procedures and antibiotic therapy and the patient has been followed up for 2 years without recurrence. Clinicians should be vigilant about the possibility of necrotizing fasciitis in patients with erythema, pain, rapid ulceration of skin and soft tissue, particularly in immunocompromised individuals with long-term use of immunosuppressive agents. It is crucial for saving life by early multi-disciplinary consultation, prompt diagnosis, and aggressive treatment.
PubMed: 38234694
DOI: 10.2147/CCID.S443374 -
Revista Espanola de Enfermedades... Jan 2024Background Pyoderma gangrenosum (PG) is a rare extraintestinal manifestation of inflammatory bowel disease. In recent years, the use of biologics in PG has been on the...
Background Pyoderma gangrenosum (PG) is a rare extraintestinal manifestation of inflammatory bowel disease. In recent years, the use of biologics in PG has been on the rise and has shown promising results. The surgical treatment of PG remains a topic of debate, with limited reports on the use of postoperative biologic therapy. Case reprt: This case report describes a 52-year-old woman who presented with multiple skin ulcers, pus discharge, and bloody diarrhea. The patient was diagnosed with PG with ulcerative colitis based on medical history, ulcer appearance, histopathology, treatment response, and the presence of ulcerative colitis. Surgical intervention was performed to repair the ulcers and amputate the fourth finger and fourth toe of both feet. Additionally, infliximab induction therapy was initiated two weeks after the surgery. The patient's intestinal symptoms demonstrated improvement, and after 10 months of treatment, the lesions were completely healed with no recurrence of skin ulcers. Conclusions This case report highlights a rare instance of successful treatment for PG with ulcerative colitis through a combination of surgery and postoperative infliximab.
PubMed: 38205697
DOI: 10.17235/reed.2024.10144/2023 -
Clinical, Cosmetic and Investigational... 2024Janus kinase (JAK) inhibitors are increasingly being used in dermatology due to their broad potential in managing both local and systemic inflammation. More recently,...
Janus kinase (JAK) inhibitors are increasingly being used in dermatology due to their broad potential in managing both local and systemic inflammation. More recently, abrocitinib, an oral JAK 1 inhibitor, has shown promising clinical efficacy in the treatment of various skin disorders beyond moderate to severe atopic dermatitis (AD). We firstly presented three cases, each with diagnosis of pyoderma gangrenosum (PG), livedoid vasculopathy (LV), or hidradenitis suppurativa (HS), and conducted a comprehensive scoping review of the available literature on the use of abrocitinib in the treatment of diverse skin disorders. We summarized a total of 16 skin disorders, including our cases. The results indicated that abrocitinib, whether used as monotherapy or in combination with other treatments, was effective and well-tolerated in these disorders. These findings expanded the range of diseases for which abrocitinib may serve as an alternative therapeutic choice.
PubMed: 38204456
DOI: 10.2147/CCID.S446369 -
Dermatology Reports Dec 2023
PubMed: 38196898
DOI: 10.4081/dr.2023.9633 -
Cell Death Discovery Jan 2024Neutrophils have both antimicrobial ability and pathogenic effect in the immune system, neutrophil extracellular traps (NETs) formation is one of the representative... (Review)
Review
Neutrophils have both antimicrobial ability and pathogenic effect in the immune system, neutrophil extracellular traps (NETs) formation is one of the representative behaviors of their dual role. NETs formation was triggered by pathogen-related components and pathogen non-related proteins as cytokines to exert its effector functions. Recent studies indicate that the pathogenicity of NETs contributed to several skin diseases such as psoriasis, Stevens-Johnson syndrome, toxic epidermal necrolysis, and neutrophilic dermatosis. Especially in neutrophilic dermatosis, a heterogeneous group of inflammatory skin disorders characterized with sterile neutrophilic infiltrate on dermis, NETs formation was reported as the way of participation of neutrophils in the pathogenesis of these diseases. In this review, we describe the different processes of NETs formation, then summarized the most recent updates about the pathogenesis of neutrophilic dermatosis and the participation of NETs, including pyoderma gangrenosum and PAPA syndrome, Behçet syndrome, hidradenitis suppurativa, Sweet Syndrome, pustular dermatosis and other neutrophilic dermatosis. Furthermore, we discuss the link between NETs formation and the development of neutrophilic dermatosis.
PubMed: 38195543
DOI: 10.1038/s41420-023-01787-2 -
Journal of Clinical Medicine Research Dec 2023A 44-year-old woman who had been diagnosed with ulcerative colitis (UC) at 22 years old was diagnosed with severe flare-up of UC based on endoscopic findings associated...
A 44-year-old woman who had been diagnosed with ulcerative colitis (UC) at 22 years old was diagnosed with severe flare-up of UC based on endoscopic findings associated with new-onset active pyoderma gangrenosum (PG) on both lower legs after she decided to discontinue UC treatment. Systemic treatment with intravenous prednisolone at 30 mg/day had achieved insufficient response to UC and PG, resulting in a diagnosis of corticosteroid-refractory UC and PG. Combination therapy with upadacitinib at 45 mg/day plus intensive granulocyte and monocyte adsorptive apheresis (GMA) was started to achieve clinical remission of UC. Ten weeks after starting this combination therapy, clinical improvement of UC was achieved with PG ulcer healing on both lower legs. A combination of upadacitinib plus intensive GMA may offer an effective therapeutic option for patients with active PG in addition to UC but has yet to be approved for induction or maintenance treatment of PG worldwide. PG is a dermatological involvement in UC patients that requires attention.
PubMed: 38189038
DOI: 10.14740/jocmr5005