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Clinical Case Reports Feb 2024Pyoderma gangrenosum is a rare inflammatory ulcerative skin disease of unknown etiology. We report an image of a patient with pyoderma gangrenosum who presented right...
Pyoderma gangrenosum is a rare inflammatory ulcerative skin disease of unknown etiology. We report an image of a patient with pyoderma gangrenosum who presented right leg ulcers with violaceous margins, histologically characterized by mono- and polynuclear cell infiltrates. The patient was successfully treated with cyclosporin A.
PubMed: 38389963
DOI: 10.1002/ccr3.8446 -
Frontiers in Immunology 2023Inflammatory bowel disease (IBD) is a chronic inflammatory disease typically involving the gastrointestinal tract but not limited to it. IBD can be subdivided into... (Review)
Review
Inflammatory bowel disease (IBD) is a chronic inflammatory disease typically involving the gastrointestinal tract but not limited to it. IBD can be subdivided into Crohn's disease (CD) and ulcerative colitis (UC). Extraintestinal manifestations (EIMs) are observed in up to 47% of patients with IBD, with the most frequent reports of cutaneous manifestations. Among these, pyoderma gangrenosum (PG) and erythema nodosum (EN) are the two most common skin manifestations in IBD, and both are immune-related inflammatory skin diseases. The presence of cutaneous EIMs may either be concordant with intestinal disease activity or have an independent course. Despite some progress in research on EIMs, for instance, ectopic expression of gut-specific mucosal address cell adhesion molecule-1 (MAdCAM-1) and chemokine CCL25 on the vascular endothelium of the portal tract have been demonstrated in IBD-related primary sclerosing cholangitis (PSC), little is understood about the potential pathophysiological associations between IBD and cutaneous EIMs. Whether cutaneous EIMs are inflammatory events with a commonly shared genetic background or environmental risk factors with IBD but independent of IBD or are the result of an extraintestinal extension of intestinal inflammation, remains unclear. The review aims to provide an overview of the two most representative cutaneous manifestations of IBD, describe IBD's epidemiology, clinical characteristics, and histology, and discuss the immunopathophysiology and existing treatment strategies with biologic agents, with a focus on the potential pathophysiological associations between IBD and cutaneous EIMs.
Topics: Humans; Inflammatory Bowel Diseases; Crohn Disease; Colitis, Ulcerative; Pyoderma Gangrenosum; Erythema Nodosum
PubMed: 37954590
DOI: 10.3389/fimmu.2023.1234535 -
BMJ Case Reports Dec 2023A perimenopausal woman with abnormal uterine bleeding underwent an uncomplicated laparoscopic hysterectomy. Postoperatively, she developed fever, abdominal erythema and...
A perimenopausal woman with abnormal uterine bleeding underwent an uncomplicated laparoscopic hysterectomy. Postoperatively, she developed fever, abdominal erythema and pain. Imaging revealed diffuse abdominal wall skin thickening, most pronounced at the right port site with a small area concerning for developing abscess. There was high clinical suspicion for necrotising fasciitis due to rapidly progressive skin deterioration. Despite antibiotics and surgical debridement, her condition progressed. Biopsy of the inflamed tissue confirmed a diagnosis of pyoderma gangrenosum (PG), and treatment with daily prednisone led to rapid improvement of symptoms.Successful diagnosis and treatment of the patient's symptoms required multidisciplinary collaboration among gynaecology, general surgery and dermatology. PG, although a well-known condition among dermatologists, is rarely, if ever, encountered by gynaecologists, and its resemblance to conditions such as necrotising fasciitis complicates early detection and intervention. This case highlights the diagnostic and management challenges associated with PG in the gynaecological setting.
Topics: Female; Humans; Pyoderma Gangrenosum; Fasciitis, Necrotizing; Anti-Bacterial Agents; Skin; Gynecologic Surgical Procedures
PubMed: 38160031
DOI: 10.1136/bcr-2023-256676 -
Deutsches Arzteblatt International Sep 2020
Topics: Aged; Biopsy; Chronic Disease; Humans; Male; Pyoderma Gangrenosum
PubMed: 33263537
DOI: 10.3238/arztebl.2020.0614a -
CMAJ : Canadian Medical Association... Sep 2019
Topics: Cyclosporine; Female; Humans; Immunosuppressive Agents; Leg; Lupus Erythematosus, Systemic; Middle Aged; Pyoderma Gangrenosum
PubMed: 31548193
DOI: 10.1503/cmaj.190721 -
Veterinary Medicine and Science Nov 2022Pyoderma is a purulent skin infection usually caused by bacteria and can be divided into primary and secondary categories based on histology. In the present study, an...
Pyoderma is a purulent skin infection usually caused by bacteria and can be divided into primary and secondary categories based on histology. In the present study, an 18-month-old female mixed breed sheep was examined for pyoderma at the injection site of the enterotoxemia vaccine. After routine bacteriology and histopathology procedures, secondary pyoderma caused by Proteus mirabilis was diagnosed. The bacterium analysed using genome sequencing and new strain called AJJ 2021 was diagnosed. This is the first report of pyoderma caused by Proteus mirabilis in sheep.
Topics: Female; Sheep; Animals; Proteus mirabilis; Pyoderma; Sheep Diseases
PubMed: 36049140
DOI: 10.1002/vms3.926 -
Frontiers in Immunology 2022Interleukin (IL)-18 is a pleiotropic, pro-inflammatory cytokine involved in the regulation of innate and adaptive immune responses. IL-18 has attracted increasing... (Review)
Review
Interleukin (IL)-18 is a pleiotropic, pro-inflammatory cytokine involved in the regulation of innate and adaptive immune responses. IL-18 has attracted increasing attention as a key mediator in autoinflammatory diseases associated with the development of macrophage activation syndrome (MAS) including systemic juvenile idiopathic arthritis and adult-onset Still's disease. In these diseases, dysregulation of inflammasome activity and overproduction of IL-18 might be associated with the development of MAS by inducing natural killer cell dysfunction. Serum IL-18 levels are high in patients with these diseases and therefore are useful for the diagnosis and monitoring of disease activity. In contrast, a recent study revealed the overproduction of IL-18 was present in cases of autoinflammation without susceptibility to MAS such as pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. The pathogenic and causative roles of IL-18 remain unclear in these autoinflammatory diseases. Further investigations are necessary to clarify the role of IL-18 and its importance as a therapeutic target in the pathogenesis of autoinflammatory diseases.
Topics: Acne Vulgaris; Adult; Arthritis, Infectious; Cytokines; Hereditary Autoinflammatory Diseases; Humans; Inflammasomes; Interleukin-18; Macrophage Activation Syndrome; Pyoderma Gangrenosum
PubMed: 36211331
DOI: 10.3389/fimmu.2022.951535 -
BMJ Case Reports Jun 2021A 75-year-old woman was admitted with sepsis and treated with broad-spectrum antibiotics until examination of her lower limbs noted necrotising wounds. Surgical...
A 75-year-old woman was admitted with sepsis and treated with broad-spectrum antibiotics until examination of her lower limbs noted necrotising wounds. Surgical intervention was advised by the plastic surgeons; however, she was deemed unsuitable for intensive care. She underwent incision and drainage of the necrotic area and biopsies were taken. She deteriorated clinically and the decision was made for best supportive care and was therefore transferred to the inpatient palliative care unit for end-of-life care. However, she stabilised, and based on culture sensitivities, antibiotics were restarted. It was also noted that the patient had a 3-month history of loose stools, which had not been addressed previously. The biopsies were suggestive of pyoderma gangrenosum, prompting a dermatology review, and prednisolone and doxycycline were started. The wounds and her loose stools improved, and with ongoing rehabilitation, she made a full recovery. Referral to gastroenterology was made.
Topics: Aged; Biopsy; Diagnosis, Differential; Drainage; Female; Humans; Prednisolone; Pyoderma Gangrenosum
PubMed: 34099445
DOI: 10.1136/bcr-2020-240133 -
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 -
Dermatology (Basel, Switzerland) 2022The International Dermatology Outcome Measures (IDEOM) initiative is a non-profit organization that aims to develop evidence-based outcome measurements to evaluate the... (Review)
Review
BACKGROUND
The International Dermatology Outcome Measures (IDEOM) initiative is a non-profit organization that aims to develop evidence-based outcome measurements to evaluate the impact of treatments for patients with dermatological disease. IDEOM includes all key stakeholders in dermatology (patient, physician, industry, insurer, and government) during the process of developing such outcome measurements.
SUMMARY
Here, we provide an update of IDEOM activities that were presented at the 2020 IDEOM Virtual Annual Meeting (October 23-24, 2020). During the meeting, multiple IDEOM workgroups (psoriasis, psoriatic arthritis, hidradenitis suppurativa, acne, pyoderma gangrenosum, and actinic keratosis) shared their progress to date, as well as future directions in developing and validating Patient-Reported Outcome Measures. Updates on demonstrating efficacy in clinicals trials by the US Food and Drug Administration are also summarized.
KEY MESSAGES
In this report, we summarize the work presented by each IDEOM workgroup (psoriasis, psoriatic arthritis, hidradenitis suppurativa, acne, pyoderma gangrenosum, and actinic keratosis) at the 2020 IDEOM Virtual Annual Meeting.
Topics: Acne Vulgaris; Arthritis, Psoriatic; Dermatology; Hidradenitis Suppurativa; Humans; Keratosis, Actinic; Outcome Assessment, Health Care; Psoriasis; Pyoderma Gangrenosum
PubMed: 34537770
DOI: 10.1159/000518966