Dysregulation of inflammatory gene expression in lesional and nonlesional skin of patients with pyoderma gangrenosum

Ortega‐Loayza, Alex G.; Nugent, William H.; Lucero, Olivia M.; Washington, Sonya L.; Nunley, Julia R.; Walsh, Scott W.

doi:10.1111/bjd.15837

Cited by 28 publications

(28 citation statements)

References 8 publications

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“…Interestingly, this study argues that IL-1 beta is not specific to PG, as it is overexpressed in other conditions which may cause skin ulcerations. This notion is further supported by the fact that not all PG lesions respond to interleukin-1 inhibitors [18].…”

Section: Pathophysiologymentioning

confidence: 89%

Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

Skopis

Bag-Ozbek

2021

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Pyoderma gangrenosum (PG) is a rare entity that is characterized by infiltration of neutrophils into the dermis, causing the formation of rapidly enlarging, painful and necrotic skin ulcers. The pathophysiology of PG is still poorly understood. However, genetic, autoimmune and autoinflammatory mechanisms have been proposed that could potentially explain the etiology of this ulcerating skin disorder. Early diagnosis and treatment are key, as the disease course is rapidly progressive and can leave disfiguring, cribriform scars. However, the diagnosis of PG proves difficult, firstly because there are multiple variants of the disease and secondly because it is a clinical diagnosis and can appear similar to that of other diseases such as vasculitis, skin/soft tissue infections and malignancy. Additionally, there are no official diagnostic criteria to aid in the recognition of PG, which often leads to significant delays in diagnosis. The treatment of PG consists in immunosuppression. However, due to a lack of standardized guidelines, therapeutic regimens are usually dependent upon the individual clinician’s experience and are based on little evidence. Knowledge of the clinical features and pathophysiology of PG can aid in early diagnosis and targeted treatment strategies, which in turn results in improved patient outcomes.

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Section: Pathophysiologymentioning

confidence: 89%

Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

Skopis

Bag-Ozbek

2021

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“…[ 27 ] Overexpression of some Pattern Recognition Receptors, like Toll-like receptors, has also been recently reported in lesional skin. [ 28 ]…”

Section: Discussionmentioning

confidence: 99%

Two case reports of pyoderma gangrenosum and systemic lupus erythematosus

et al. 2018

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Rationale:Pyoderma gangrenosum (PG), like other neutrophilic dermatosis, may be associated with a variety of systemic disorders including inflammatory bowel diseases, rheumatoid arthritis, and hematologic disorders. Conversely, the association between PG and systemic lupus erythematosus (SLE) has rarely been reported.Patient concerns:We report here 2 cases of this association.Diagnoses:The first case involves a 32-year-old woman who developed, 1 year after SLE diagnosis, 3 painful nodular lesions of PG on her face, and cervical area. The second case was observed in a 37-year-old woman referred for ulcerative nodular papules of PG on her legs, whereas she had been diagnosed with SLE 10 years before. SLE was inactive in the first case, whereas PG occurred during a lupus flare up in the second one.Interventions:We found 23 previous cases of SLE and PG in the literature with most cases (12/20) occurring during a lupus flare.Outcomes:Although rare, this association may be supported by common innate immunity dysregulation and abnormal neutrophil activation.Lessons:PG and other neutrophilic diseases reported in patients with SLE may be added to the large clinical spectrum of cutaneous lesions observed in SLE.

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“…Current research is continuing to identify new—and confirm already-known—cytokines and signaling cascades involved in the pathogenesis of PG. The innate immune system signaling pathways, pattern recognition receptor (PRR) pathways (which are associated with autoimmune diseases such as IBD and RA), and Janus kinase (JAK) 1–3 and signal transducer and activator of transcription (STAT) pathways were upregulated in lesional skin compared with non-lesional skin in patients with PG 15, 16 . In comparisons of lesional skin of individuals with classic ulcerative PG and PG, acne, and suppurative hidradenitis (PASH) syndrome, both showed overexpression of IL-1β, tumor necrosis factor alpha (TNFα), IL-17, endothelial- and leukocyte-selectin, and chemokines IL-8, CXCL16, and RANTES 17, 18 .…”

Section: Pathophysiologymentioning

confidence: 99%

Recent advances in managing and understanding pyoderma gangrenosum

2019

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Pyoderma Gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. PG has historically been a challenging disorder to diagnose, leading to the development of new diagnostic criteria rather than the traditional approach of a diagnosis of exclusion. The pathophysiology is thought to involve both innate and adaptive immune system dysregulation, neutrophilic abnormalities, environmental, and genetic factors. As of today, no gold standard therapy exists for the treatment of PG, and the literature is restricted to mainly case reports, case series, and 2 small randomized clinical trials. Topical, systemic, and biologic therapy, as well as adequate analgesia and proper wound care all play a role in the management of PG. Recent studies have identified additional cytokines and signalling cascades thought to be involved in the pathogenesis of PG, ultimately leading to the development of new targeted therapies. This review will focus on recent advances in the pathophysiology, clinical presentation and associated comorbidities, diagnosis, and management of PG.

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Dysregulation of inflammatory gene expression in lesional and nonlesional skin of patients with pyoderma gangrenosum

Cited by 28 publications

References 8 publications

Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

Two case reports of pyoderma gangrenosum and systemic lupus erythematosus

Recent advances in managing and understanding pyoderma gangrenosum

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