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Journal of Clinical Immunology Dec 2023Pyrin is a cytosolic protein encoded by the MEFV gene, predominantly expressed in innate immune cells. Upon activation, it forms an inflammasome, a multimolecular... (Review)
Review
Pyrin is a cytosolic protein encoded by the MEFV gene, predominantly expressed in innate immune cells. Upon activation, it forms an inflammasome, a multimolecular complex that enables the activation and secretion of IL-1β and IL-18. In addition, the Pyrin inflammasome activates Gasdermin D leading to pyroptosis, a highly pro-inflammatory cell death. Four autoinflammatory syndromes are associated with Pyrin inflammasome dysregulation: familial Mediterranean fever, hyper IgD syndrome/mevalonate kinase deficiency, pyrin-associated autoinflammation with neutrophilic dermatosis, and pyogenic arthritis, pyoderma gangrenosum, and acne syndrome. In this review, we discuss recent advances in understanding the molecular mechanisms regulating the two-step model of Pyrin inflammasome activation. Based on these insights, we discuss current pharmacological options and identify a series of existing molecules with therapeutic potential for the treatment of pyrin-associated autoinflammatory syndromes.
Topics: Humans; Inflammasomes; Pyrin; Familial Mediterranean Fever; Syndrome; Pyoderma Gangrenosum; Mevalonate Kinase Deficiency
PubMed: 38129719
DOI: 10.1007/s10875-023-01621-5 -
Dermatologic Clinics Apr 2024Neutrophilic dermatoses (NDs) encompass a wide range of cutaneous and extracutaneous manifestations, many of which impair quality of life (QoL) and are difficult to... (Review)
Review
Neutrophilic dermatoses (NDs) encompass a wide range of cutaneous and extracutaneous manifestations, many of which impair quality of life (QoL) and are difficult to treat. Although NDs are transient and mild, others are chronic, severely debilitating conditions with profound impacts on QoL, including pain, mental health, occupational limitations, and sexual health implications. Current literature lacks attention to these unique care challenges to the ND patient population. The authors aim to summarize what is currently known about QoL in NDs and identify which diseases would benefit from additional research and disease-specific QoL assessment.
Topics: Humans; Pyoderma Gangrenosum; Quality of Life; Neutrophils; Skin; Sweet Syndrome
PubMed: 38423691
DOI: 10.1016/j.det.2023.08.011 -
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 -
Veterinary Pathology Nov 2023The skin covers the external surface of animals, and it is constantly exposed to and inhabited by different microorganisms, including bacteria. Alterations in the skin... (Review)
Review
The skin covers the external surface of animals, and it is constantly exposed to and inhabited by different microorganisms, including bacteria. Alterations in the skin barrier allow commensal and/or pathogenic bacteria to proliferate and penetrate deep into the lower layers of the skin. Being the first barrier to the external environment, the skin is prone to injuries, allowing the penetration of microorganisms that may lead to severe deep infections. Companion animals, especially dogs, are prone to bacterial infections, often secondary to allergic dermatitis. When environmental conditions are unfavorable, horses, cattle, sheep, and goats can develop superficial infections, such as those caused by . Deep inflammation is commonly caused by spp., which results in granulomatous to pyogranulomatous dermatitis and panniculitis. Likewise, bacteria such as spp. and spp. can cause deep pyogranulomatous inflammation. Bacteria that lead to deep necrotizing lesions (eg, necrotizing fasciitis/flesh-eating bacteria) can be severe and even result in death. This review includes an overview of the most common cutaneous bacterial infections of domestic animals, highlighting the main features and histologic morphology of the bacteria, cutaneous structures involved, and the type of inflammatory infiltrates.
Topics: Animals; Dogs; Horses; Cattle; Sheep; Skin; Dermatitis; Animals, Domestic; Panniculitis; Inflammation; Cattle Diseases; Dog Diseases; Horse Diseases; Sheep Diseases
PubMed: 37264789
DOI: 10.1177/03009858231176558 -
Advances in Skin & Wound Care Feb 2024Wound debridement improves healing in a variety of acute and chronic ulcers. However, there is concern that debridement may trigger pathergy and worsen pyoderma...
BACKGROUND
Wound debridement improves healing in a variety of acute and chronic ulcers. However, there is concern that debridement may trigger pathergy and worsen pyoderma gangrenosum (PG).
OBJECTIVE
To determine whether published evidence supports conservative wound debridement for PG.
DATA SOURCES
The authors reviewed the literature published in MEDLINE through January 2023 using the search germs "pyoderma gangrenosum" and "debridement."
STUDY SELECTION
Articles reporting sharp surgical debridement or maggot debridement for PG were included in the review. The authors also searched the reference sections of the reviewed articles for additional reports on debridement for PG.
DATA EXTRACTION
Clinical data regarding patient status, procedures performed, and patient outcomes were extracted from the selected articles.
DATA SYNTHESIS
There are multiple reports of uncontrolled, active-phase PG wounds worsening after aggressive excisional debridement of viable inflamed tissues. In contrast, there is no evidence indicating that conservative debridement of nonviable necrotic tissue worsens PG wounds, regardless of the disease activity. There are multiple reports of successful debridement and surgical grafting for PG in remission.
CONCLUSIONS
There is no evidence in favor of or against using conservative debridement of nonviable necrotic tissue for a PG wound. Therefore, it should not be considered contraindicated, even in the active phase of the disease.
Topics: Animals; Humans; Debridement; Pyoderma Gangrenosum; Wound Healing; Larva; Necrosis
PubMed: 38241454
DOI: 10.1097/ASW.0000000000000092 -
Revista Espanola de Enfermedades... Aug 2023Peristomal ulcer is a frequent complication that, in most cases, is mild and responds to topical treatment. In much less frequent cases, it is associated with other...
Peristomal ulcer is a frequent complication that, in most cases, is mild and responds to topical treatment. In much less frequent cases, it is associated with other pathologies, such as Crohn's disease, requiring in these cases systemic treatment, including surgery. The differential diagnosis between Cutaneous Inflammatory Bowel Disease and Pyoderma Gangrenosum is interesting, although both are managed in a similar way.
Topics: Humans; Crohn Disease; Inflammatory Bowel Diseases; Pyoderma Gangrenosum; Administration, Topical; Wound Healing
PubMed: 35791785
DOI: 10.17235/reed.2022.8909/2022 -
Dermatologic Clinics Apr 2024Postoperative pyoderma gangrenosum and peristomal pyoderma gangrenosum are 2 subtypes of pyoderma gangrenosum. The diagnosis is made as a clinicopathologic correlation... (Review)
Review
Postoperative pyoderma gangrenosum and peristomal pyoderma gangrenosum are 2 subtypes of pyoderma gangrenosum. The diagnosis is made as a clinicopathologic correlation when assessing a rapidly progressing ulcer with irregular and undermined borders following a surgical procedure, trauma, or the creation of a stoma. Familiarity with the associated risk factors and distinguishing features of these disorders can facilitate prompt recognition, proper diagnosis, and the initiation of treatment. Management usually involves the use of corticosteroids and steroid-sparing agents as immunomodulators to shift the inflammatory neutrophilic dermatoses to chronic noninflammatory wounds and eventual healing.
Topics: Humans; Pyoderma Gangrenosum; Adrenal Cortex Hormones; Wound Healing; Risk Factors
PubMed: 38423679
DOI: 10.1016/j.det.2023.12.001 -
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 -
Systemic calcineurin inhibitors tacrolimus and voclosporin: A review of off-label dermatologic uses.Journal of the American Academy of... Feb 2024Systemic calcineurin inhibitors, cyclosporine, tacrolimus, and voclosporin, have been utilized in various dermatologic conditions. Although there have been numerous... (Review)
Review
BACKGROUND
Systemic calcineurin inhibitors, cyclosporine, tacrolimus, and voclosporin, have been utilized in various dermatologic conditions. Although there have been numerous off-label dermatologic indications with published guidelines for cyclosporine, there is no established strong consensus for tacrolimus and voclosporin.
OBJECTIVE
To conduct a review of off-label use of systemic tacrolimus and voclosporin in various dermatoses to better inform treatment methods.
METHODS
A literature search was conducted using PubMed and Google Scholar. Relevant clinical trials, observational studies, case series, and reports regarding off-label dermatologic uses of systemic tacrolimus and voclosporin were included.
RESULTS
Tacrolimus shows promise for numerous dermatologic conditions, including psoriasis, atopic dermatitis/eczema, pyoderma gangrenosum, chronic urticaria, and Behcet's disease. Randomized controlled trial data are only available for voclosporin in psoriasis, which showed efficacy but did not meet noninferiority to cyclosporine.
LIMITATIONS
Data were limited and extracted from published papers. Studies differed in methodology, and nonstandardized outcomes limited the conclusions drawn.
CONCLUSIONS
In comparison to cyclosporine, tacrolimus can be considered for treatment-refractory disease or in patients with cardiovascular risk factors or inflammatory bowel disease. Voclosporin has only been utilized in psoriasis currently, and clinical trials in psoriasis show voclosporin's efficacy. Voclosporin can be considered for patients with lupus nephritis.
Topics: Humans; Calcineurin Inhibitors; Tacrolimus; Off-Label Use; Cyclosporine; Psoriasis; Immunosuppressive Agents
PubMed: 37307993
DOI: 10.1016/j.jaad.2023.05.074 -
Archives of Dermatological Research Sep 2023Drainage from chronic wounds can significantly negatively impact a patient's quality of life. Change in severity of wound drainage is an important measure of treatment... (Review)
Review
Drainage from chronic wounds can significantly negatively impact a patient's quality of life. Change in severity of wound drainage is an important measure of treatment efficacy for wounds. This study reviews existing tools used to assess wound drainage. Qualitative drainage tools are overall less burdensome, and however, differences in user interpretation may reduce inter-rater reliability. Quantitative drainage tools enable more reliable comparisons of drainage severity and treatment response between patients but sometimes require equipment to administer, increasing responder burden. Gaps in the current wound drainage measurement landscape are highlighted. Many of the existing scales have not been validated in robust studies. There is also a lack of validated global drainage measurement tools for patients with chronic inflammatory skin disorders with drainage, such as hidradenitis suppurativa or pyoderma gangrenosum. Development of a succinct drainage measurement tool for inflammatory skin diseases where drainage is a prominent symptom will improve monitoring of meaningful treatment response.
Topics: Humans; Quality of Life; Reproducibility of Results; Hidradenitis Suppurativa; Drainage; Treatment Outcome
PubMed: 36680593
DOI: 10.1007/s00403-023-02525-5