Materiały źródłowe

• #1 Pyoderma gangrenosum | Altru Health System

  https://www.altru.org/health-library/conditions/pyoderma-gangrenosum

  Pyoderma gangrenosum (pie-o-DUR-muh gang-ruh-NO-sum) is a rare condition that causes large, painful sores on the skin. The exact causes of pyoderma gangrenosum are unknown, but it appears to be a disorder of the immune system. […] No one knows the exact cause of pyoderma gangrenosum. It’s often seen in people who have autoimmune diseases, such as ulcerative colitis, Crohn’s disease and arthritis. […] If you have pyoderma gangrenosum, getting a cut or other skin wound can bring on new sores. The condition isn’t an infection and it isn’t contagious.

• #2 Pyoderma Gangrenosum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK482223/

  Pyoderma gangrenosum is an ulcerative disorder that falls into the category of neutrophilic dermatoses. […] Despite its name, pyoderma gangrenosum is not caused by infection or gangrene. […] The most common associated systemic disorders include rheumatoid arthritis, inflammatory bowel disease, and other autoimmune and inflammatory conditions. […] In addition, pyoderma gangrenosum has an association with both solid tumors and hematologic malignancies. […] Pyoderma gangrenosum is associated with ulcerative colitis in 5% to 12% of cases and is associated with Crohn disease in 1% to 2% of cases. […] It is not clear if the development of pyoderma gangrenosum correlates with severity or flares of inflammatory bowel disease. […] Furthermore, pyoderma gangrenosum does not always resolve when the associated bowel disease is treated.

• #3 Pyoderma Gangrenosum – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/pyoderma-gangrenosum

  Pyoderma gangrenosum is a chronic inflammatory skin disorder of unknown cause that causes large sores on the skin. […] The cause is not known, but this disorder can develop after an injury or in people with certain disorders. […] The cause of pyoderma gangrenosum is unknown, but people who have pyoderma gangrenosum tend to have certain underlying disorders, including inflammatory bowel disease, rheumatoid arthritis, cancers, and blood disorders. […] In pyoderma gangrenosum, the immune system seems to be reacting to the skin itself. […] Pyoderma gangrenosum can develop on areas of skin that have recently been injured or operated on.

• #4 Pyoderma Gangrenosum – Creative Biolabs

  https://www.creative-biolabs.com/complement-therapeutics/pyoderma-gangrenosum.htm

  Pyoderma gangrenosum (PG) is an uncommon, ulcerative cutaneous condition that causes tissue to become necrotic, leading to deep ulcers on the skin, most common on legs. […] The etiology of pyoderma gangrenosum is incompletely understood, but dysregulation of the immune system (abnormal chemotaxis, neutrophil migration, phagocytosis, bactericidal ability, and abnormal neutrophil trafficking) is believed to be involved. Furthermore, genetic alternation also contributes to the pathophysiology of PG. […] So neutrophils dysfunction plays an assignable role in the etiology of PG. […] Elevated levels of inflammatory mediators have been found in lesions of PG, suggesting a pathological relationship between abnormal immune reactions and PG. […] Pyoderma gangrenosum has been reported in association with congenital complement deficiencies, especially the deficiencies of C2, C4, and C7.

• #5 Pyoderma gangrenosum: a review of the clinical, mechanistic and therapeutic landscape :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-30-number-1/pyoderma-gangrenosum-review-clinical-mechanistic-and-therapeutic-landscape

  Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that is uncommon and can sometimes be associated with systemic diseases. […] The pathophysiology underlying PG is yet to be known, although it may occur secondary to other inflammatory diseases. PG was previously thought to arise from a functional disorder of neutrophils, although recent advances suggest there is also adaptive and innate immune system dysregulation, as well as local cutaneous abnormalities. […] Approximately 25-50% of patients with PG will have an underlying inflammatory systemic disease. The systemic disease may be subclinical, and may occur before or after the onset of PG. […] The exact mechanism through which PG arises remains poorly understood. […] The rapidly progressive and ulcerative nature of this disease from seemingly normal skin makes it difficult to identify early initiating events that may lead to downstream inflammatory cascades.

• #6 Pyoderma gangrenosum: a review of the clinical, mechanistic and therapeutic landscape :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-30-number-1/pyoderma-gangrenosum-review-clinical-mechanistic-and-therapeutic-landscape

  Histologically, neutrophils are known to predominate within established PG, but it is unknown whether their presence is due to primary neutrophilic abnormalities or is secondary to an already established complex immunological dysfunction. […] The Th17 pathway has been strongly associated with PG activity. […] It is possible that Th17 may also be activated through the release of IL9 from local Th9 cells (as seen with other inflammatory dermatoses), in addition to IL23 pathways. […] The increased Th1, Th2 and Th17 response coupled with reduced Treg activity indicates that T-cell-mediated inflammation plays a substantial role in PG pathogenesis.

• #7 Pyoderma Gangrenosum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK482223/

  The pathogenesis of pyoderma gangrenosum is not fully understood. […] It is thought to involve genetic mutations, neutrophil dysfunction, and immune/inflammatory dysregulation. […] Some cases of pyoderma gangrenosum are associated with a mutation in Janus kinase 2, which is involved in the production of several cytokines. […] Abnormal cytokine signaling by T cells and macrophages is likely a component of the disease process. […] Lesions of pyoderma gangrenosum have been found to have increased levels of inflammatory mediators.

• #8 The challenges of managing patients with pyoderma gangrenosum: three case reports :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-24-no-1/challenges-managing-patients-pyoderma-gangrenosum-three-case-reports

  Pyoderma gangrenosum (PG), although first described nearly 100 years ago, remains challenging for clinicians. The aetiology of PG remains a mystery. […] Although first described almost 100 years ago, the aetiology remains a mystery. […] The physiological process that leads to PG remains ambiguous. Early hypotheses included occult bacterial infection, circulating antibodies, or the Shwartzman reaction (an immune response to bacterial endotoxins leading to tissue necrosis). However, there is a lack of evidence to support these theories. Current postulations regarding the pathogenesis of PG include neutrophil dysfunction, genetic factors and dysregulation of the innate immune system. […] Genetic features that have been reported include familial cases of PG and also PG related to pyogenic sterile arthritis syndrome (PAPA) and acne. There is also a growing body of evidence to suggest that dysregulation of the innate immune system is associated with PG. […] It has been estimated that approximately 40-50% cases of PG are associated with a systemic disease and the remainder are idiopathic.

• #9 Orphanet: Pyoderma gangrenosum

  https://www.orpha.net/en/disease/detail/48104

  A rare inflammatory neutrophilic dermatosis characterized by painful cutaneous ulcerations with a violaceous and undermined border affecting the lower extremities; however, any hair-bearing area can be affected. […] The etiology has not yet been clearly determined. […] PG can occur spontaneously or in association with other autoimmune conditions, including most commonly inflammatory bowel disease (IBD), rheumatologic conditions, hematologic disorders, or in association with autoinflammatory syndromes including PAPA (pyogenic arthritis, PG, and acne), PASH (PG, acne, and suppurative hidradenitis), PAPASH (pyogenic arthritis, acne, PG, and suppurative hidradenitis), PAC (PG, acne, and ulcerative colitis), PsAPASH (psoriatic arthritis, PG, acne and suppurative hidradenitis), PAMI (PSTPIP1-associated myeloid related proteinemia syndrome), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis).

• #10 Pyoderma gangrenosum: challenges and solutions | CCID

  https://www.dovepress.com/pyoderma-gangrenosum-challenges-and-solutions-peer-reviewed-fulltext-article-CCID

  The similarities between PG and neutrophilic diseases suggest that underlying common inflammatory pathways probably converge to their pathophysiology, leading to abnormalities in polymorphonuclear neutrophils (PMN) recruitment or homeostasis. […] Genetic abnormalities have been found in patients with PG in the context of PAPA, PAPASH (initially described as PASS pyogenic arthritis, PG, acne, and hidradenitis suppurativa), and PASH (PG, acne, and hidradenitis suppurativa) syndromes in genes that code for an aberrant production of IL1, namely PSTPIP1 gene, or in its promoters. […] Rarely, PG appears during the treatment with infliximab, etanercept, or G-CSF. Nevertheless, most reports confirm the benefit of therapy with the different TNF inhibitors, which further support an underlying immunologic mechanism in PG pathogenesis.

• #11 Pyoderma gangrenosum – Wikipedia

  https://en.wikipedia.org/wiki/Pyoderma_gangrenosum

  Pyoderma gangrenosum is a rare, inflammatory skin disease where painful pustules or nodules become ulcers that progressively grow. Pyoderma gangrenosum is not infectious. […] Though the cause is not well understood, the disease is thought to be due to immune system dysfunction, and particularly improper functioning of neutrophils. In support of an immune cause, a variety of immune mediators such as interleukin (IL)-8, IL-1, IL-6, interferon (IFN)-, granulocyte colony-stimulating factor, tumor necrosis factor alpha, matrix metalloproteinase (MMP)-9, MMP10, and elafin have all been reported to be elevated in patients with pyoderma gangrenosum. […] Also in support of an immune cause is the finding that at least half of all pyoderma gangrenosum patients suffer from immune-mediated diseases. For instance, ulcerative colitis, rheumatoid arthritis, and monoclonal gammopathies have all been associated with pyoderma gangrenosum. It can also be part of autoinflammatory syndromes such as PAPA syndrome. […] One hallmark of pyoderma gangrenosum is pathergy, which is the appearance of new lesions at sites of trauma, including surgical wounds.

• #12 Pyoderma Gangrenosum – Dermatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.856.5.

  Pyoderma gangrenosum (PG) is caused by genetic alterations of the immune system (both innate and adaptive), leading to inflammasome activation, cytokine production, and neutrophilic infiltration. […] Genetic mutations, neutrophil dysfunction, and abnormal inflammation contribute to the pathogenesis and clinical manifestations of PG. […] Underlying diseases such as IBD, rheumatoid arthritis, and hematologic disorders share similar proinflammatory pathogeneses with PG and are seen in up to 75% of PG cases. […] These PG-associated genetic syndromes share proinflammatory pathways and/or molecules (eg, interleukin 1 [IL-1]) underlying the pathogenesis of PG and associated conditions, such as IBD and psoriasis.

• #13 Is Pyoderma Gangrenosum Caused By Ulcerative Colitis?

  https://www.healthline.com/health/ulcerative-colitis/pyoderma-gangrenosum-ulcerative-colitis

  Pyoderma gangrenosum is a rare symptom of inflammatory bowel disease that causes lesions to appear on your skin. […] Doctors are still researching the connections and causes between ulcerative colitis and pyoderma gangrenosum. […] One study estimated that 2% of people with ulcerative colitis have pyoderma gangrenosum. […] What this means is that ulcerative colitis does increase the likelihood you could experience pyoderma gangrenosum. However, having ulcerative colitis doesnt mean youll get pyoderma gangrenosum, as only a small percentage of those with UC experience this skin complication. […] Doctors dont know exactly why pyoderma gangrenosum occurs, but they do think the condition is likely an autoimmune disorder. […] Their current theory is that pyoderma gangrenosum is likely an autoimmune disorder that causes your body to attack healthy tissue, leading to inflammation. […] About 50% of people with pyoderma gangrenosum have another related disorder, such as ulcerative colitis, Crohns disease, or rheumatoid arthritis.

• #14 Pyoderma Gangrenosum: Causes, Symptoms & Treatments

  https://my.clevelandclinic.org/health/diseases/17825-pyoderma-gangrenosum-pg

  Pyoderma gangrenosum is a skin condition linked to certain autoimmune diseases or an earlier skin injury. Medical experts aren’t sure of its causes, and it’s difficult to diagnose because it looks like other diseases. […] Pyoderma gangrenosum may be an autoimmune disease. That means that pyoderma gangrenosum results from your immune system damaging tissue in your own body. For some people, pyoderma gangrenosum will improve after treatment for their other systemic diseases. […] Medical experts don’t know exactly what causes pyoderma gangrenosum. More than half of people with pyoderma gangrenosum have other conditions, including rheumatoid arthritis, leukemia, lymphoma, ulcerative colitis and Crohn’s disease. […] In addition, pyoderma gangrenosum often develops after an injury to your skin (pathergy), like trauma or surgery. Sometimes, pyoderma gangrenosum appears near a surgical opening (stoma site). This is peristomal pyoderma gangrenosum. […] Like ecthyma gangrenosum, pyoderma gangrenosum also causes skin lesions. Medical experts aren’t sure what causes pyoderma gangrenosum. Autoimmune diseases may cause pyoderma gangrenosum, but pyoderma gangrenosum doesn’t primarily affect those who are immunocompromised.

• #15 Pyoderma gangrenosum

  https://www.pcds.org.uk/clinical-guidance/pyoderma-gangrenosum

  Pyoderma gangrenosum (PG) is a rare, neutrophilic dermatosis, commonly associated with systemic disease. […] 50-70% of cases have an underlying cause, approximately equally divided between three groups: Inflammatory bowel disease (IBD), especially ulcerative colitis; Haematological conditions, especially myeloid leukaemia’s; Inflammatory arthritis such as rheumatoid arthritis. […] Additional causes include chronic active hepatitis and collagen vascular disorders. […] A diagnosis may be possible based on the clinical appearance, although it is important to exclude other causes of ulcers including those secondary to infection, and factitious disease. […] Skin biopsies, taken from the edge of the lesion, may be required to exclude other conditions. Samples should be sent for both for histopathology and culture, the latter to exclude infections such as unusual fungi and atypical mycobacteria. […] Although PG characteristically results in a neutrophilic infiltrate, this is not always present, and the histopathological findings are often variable and non-specific.

• #16 Pyoderma Gangrenosum – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/pyoderma-gangrenosum

  Pyoderma gangrenosum is a chronic, neutrophilic, progressive skin necrosis of unknown etiology often associated with systemic illness and sometimes skin injury. […] Etiology of pyoderma gangrenosum is unknown, but it can be associated with various systemic illnesses, including inflammatory bowel disease, rheumatoid arthritis, cancers, and hematologic disorders (eg, monoclonal gammopathy of undetermined significance, myelodysplastic syndrome, polycythemia vera). It is thought to be mediated by an abnormal immune response. […] Pyoderma gangrenosum is often associated with a systemic disorder and is probably immune-mediated.

• #17 Pyoderma Gangrenosum: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pyoderma-gangrenosum-pro

  Pyoderma gangrenosum is an uncommon neutrophilic dermatosis that usually presents with rapidly growing, painful, undermined, and purulent ulcers that are more likely to develop at areas of trauma. […] The cause is unknown. 50-70% of cases are associated with other diseases, mainly inflammatory bowel disease (IBD), arthritis and lymphoproliferative disorders. […] PG may occur in sites of trauma; this phenomenon is called pathergy. It often begins in sites of minor injury (reported in 20-30% of cases). […] The occurrence of PG does not seem to relate to the disease activity in conditions such as IBD and arthritis. […] Possible precipitating factors for PG are: Biopsies, intradermal skin testing, injections, insect bites, etc (due to pathergy). […] The clinical course is variable and difficult to predict. There may be spontaneous resolution, a quiescent phase for months or years or flare-ups following minimal trauma or for no apparent cause.

• #18 Pyoderma Gangrenosum – Best Wound Practice

  https://bestwoundpractice.com/pyoderma-gangrenosum-2/

  Pyoderma Gangrenosum (PG) is frequently underrecognized in clinical practice, and there is often a significant delay prior to establishing PG as the diagnosis and starting appropriate therapy. […] PG is associated with other autoimmune/systemic conditions in about 50% of patients, especially inflammatory bowel disease in about 30% of cases (e.g. Crohns Disease and Ulcerative Colitis), as well as seronegative arthritis. […] Another key learning point from this case regards the Pathergy phenomenon, which describes an acute worsening of a wound after mechanical trauma. This can be seen with both operative debridement and wound biopsy, and is a feature of Pyoderma Gangrenosum as well as the ulcers prominent in Behcet Syndrome (an autoimmune vasculitis disorder with prominent muco-cutaneous ulcers). Pathergy is originally described in association with the Pathergy Test, in which the skin is pricked with a 20 gauge needle, and the eruption of a papular or pustular lesion results at the test site within 48 hours in a positive test. This test is rarely performed as it was initially described, and so the term Pathergy, is now used colloquially by many physicians to describe an abrupt worsening of a wound after biopsy, debridement or surgery. In the above case, the suggestive feature of pathergy was purulence and skin edge necrosis after her surgical resection.

• #19 Pyoderma Gangrenosum Causes, Treatments, & Misdiagnosis

  https://www.epiphanydermatology.com/medical-dermatology/pyoderma-gangrenosum-treatment/

  The remaining 50% are either idiopathic (no associated systemic disease) or we just haven’t found the association. […] Postoperative PG seems to occur most often in breast tissue after tumor removal but also after augmentations. Orthopedic, cardiothoracic, abdominal, and other surgeries may also initiate PG at the sites of incision.

• #20 Pyoderma Gangrenosum: Symptoms, Causes, and Treatment — DermNet

  https://dermnetnz.org/topics/pyoderma-gangrenosum

  Pyoderma gangrenosum is an autoinflammatory disease (excessive response to an internal antigen) due to some form of neutrophil dysfunction. T lymphocytes and cytokines are involved. There may be a genetic predisposition. […] Drugs are occasionally implicated as triggers of pyoderma gangrenosum, especially cocaine, isotretinoin, propylthiouracil, and sunitinib (a protein kinase inhibitor). […] Injury to the skin is a common trigger (the pathergic response), and a surgical trigger is well known, and often misinterpreted as a wound infection.

• #21 Pathology Outlines – Pyoderma gangrenosum

  https://www.pathologyoutlines.com/topic/skinnontumorpyodermagangrenosum.html

  Unknown etiology; 25 – 50% idiopathic […] Up to 50% associated with systemic disease: Inflammatory bowel disease (e.g. ulcerative colitis) […] Arthritis […] Hematological malignancy or monoclonal gammopathies […] Other associated conditions: rheumatoid arthritis, seronegative arthritis, pregnancy, viral hepatitis, HIV, granulomatosis with polyangiitis.

• #22 Pyoderma gangrenosum – a review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-2-19

  Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis. […] Aetiology has not been clearly determined yet. […] Fulbright et al. hypothesized that PG results from an aberrant immune response to yet unidentified factors. […] Since inflammatory bowel disease is the most common underlying disorder, cross-reacting antigens in the bowel and the skin could be responsible for secondary cutaneous manifestation. […] Cellular analysis in PG demonstrated aberrant integrin oscillations on neutrophils and aberrant neutrophil tracking of patients with PG. […] Pathways to protect the epidermis from neutrophil infiltration seem to be insufficient in PG resulting in tissue necrosis.

• #23 Four Types of Pyoderma Gangrenosum | Skin Diseases

  https://woundeducators.com/pyoderma-gangrenosum-etiology-and-presentation/?srsltid=AfmBOorOa1tPsBKuAxbo55z4bZg8M0OubiOxsTWk0f8SMb4isRo__6fX

  Pyoderma gangrenosum is a rare but serious ulcerating skin disease, which can present in many forms and to a variety of health professionals. This means a diagnosis of the condition is often delayed, lead to serious clinical consequences for the patient. […] The etiology of pyoderma gangrenosum is not well understood, but is thought to involve an inappropriate immune response to a number of unidentified factors. […] Since inflammatory bowel disease is the most common underlying disorder, cross-reacting antigens in the bowel and the skin could be responsible for secondary cutaneous manifestation. […] Cellular analysis has shown aberrant neutrophil activity in patients with pyoderma gangrenosum, with tissue necrosis caused by unchallenged neutrophil infiltration.

• #24 Pyoderma Gangrenosum: Background, Epidemiology, Prognosis

  https://emedicine.medscape.com/article/1123821-overview

  Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition whose cause is uncertain. Although the etiology of this condition is poorly understood, dysregulation of the immune system (specifically, altered neutrophil chemotaxis) is believed to be involved. Pyoderma gangrenosum is associated with systemic diseases in at least 50% of patients who are affected. […] The diagnosis is made by excluding other causes of similar-appearing cutaneous ulcerations, including infection, malignancy, vasculitis, vasculopathy, venous insufficiency, collagen-vascular diseases, diabetes, and trauma. […] Patients with pyoderma gangrenosum may have involvement of other organ systems that manifests as sterile neutrophilic infiltrates. Culture-negative pulmonary infiltrates are the most common extracutaneous manifestation. […] Therapy for pyoderma gangrenosum involves the use of anti-inflammatory agents, including antibiotics, corticosteroids, immunosuppressive agents, and biologic agents.

• #25 Pyoderma Gangrenosum (PG) | OHSU

  https://www.ohsu.edu/dermatology/pyoderma-gangrenosum-pg

  Pyoderma gangrenosum (PG) is a rare skin condition characterized by open sores or wounds that develop on the skin (skin ulcers). […] The exact cause of pyoderma gangrenosum is unknown, but it is likely a disorder of the immune system. […] PG is associated with other inflammatory conditions such as inflammatory bowel disease and rheumatoid arthritis; it is also sometimes associated with hematological malignancies. […] Dr. Ortega has dedicated his career to advancing PG diagnosis and treatment through research, assembling basic scientists, biostatisticians, and clinicians together to form the pyoderma gangrenosum study team (PYGAS) at OHSU. […] Dr. Ortega is leading efforts to identify specific markers that could be used to minimize the challenge of diagnosing PG worldwide, thus improve patient outcomes. […] He has also made contributions to the awareness, diagnosis and management of PG and its variants. […] He has demonstrated the unmet need for proper diagnostic tests in PG after highlighting the causes of misdiagnosis resulting in amputation.

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