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Clinical Gastroenterology and... Jul 2019
Topics: Humans; Piperidines; Pyoderma Gangrenosum; Pyrimidines; Pyrroles
PubMed: 30625401
DOI: 10.1016/j.cgh.2018.12.044 -
F1000Research 2019Pyoderma Gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. PG has historically been a... (Review)
Review
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.
Topics: Cytokines; Humans; Pyoderma Gangrenosum
PubMed: 31885859
DOI: 10.12688/f1000research.19909.1 -
Journal of Cutaneous Medicine and... 2021Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful and ulcerating lesions on the skin. It rarely involves the face and is often... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful and ulcerating lesions on the skin. It rarely involves the face and is often difficult to diagnose. There are few cases reported in the literature of PG involving the face.
AIM
To share our experience with 5 patients in whom the final diagnosis was PG involving the face, and to review the literature.
METHODS
We report a series of 5 patients with a final diagnosis of PG involving the face and reviewed relevant literature. We searched through PubMed andEMBASE using keywords such as "face" and "pyoderma gangrenosum," "blastomycosis-like pyoderma gangrenosum, vegetative pyoderma gangrenosum and granulomatous pyoderma gangrenosum."
RESULTS
We report 5 patients (4 females) with pyoderma gangrenosum involving the face. All 5 had a final diagnosis of superficial granulomatous PG. All cases presented with nonhealing facial ulcer most commonly on cheeks and a common histopathology of mixed inflammatory infiltrates, multinucleated giant cells, and plasma cells with some granulomatous inflammation.
CONCLUSIONS
PG can involve the face, and all 5 of our patients had the superficial granulomatous as the most common form.
Topics: Adolescent; Adult; Aged, 80 and over; Facial Dermatoses; Female; Humans; Male; Middle Aged; Pyoderma Gangrenosum
PubMed: 33541119
DOI: 10.1177/1203475420988864 -
Acta Dermatovenerologica Alpina,... Mar 2022Pyoderma gangrenosum is an uncommon neutrophilic dermatosis that usually presents with rapidly growing, painful, undermined, and purulent ulcers that are more likely to... (Review)
Review
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. It is associated with underlying systemic diseases in more than half of cases, most commonly with inflammatory bowel disease. Pyoderma gangrenosum has no specific clinical, histologic, or laboratory findings, and so the diagnosis is based on exclusion of all other diagnostic possibilities, especially infectious causes. Misdiagnoses are frequent, with systemic vasculitides representing one of the main imitators. Treatment of pyoderma gangrenosum usually requires a multidisciplinary approach, with infliximab emerging as the best treatment option for cases associated with inflammatory bowel disease. The prognosis of pyoderma gangrenosum remains unpredictable, and recurrences are common. Here, we report a case of mucocutaneous pyoderma gangrenosum as a preceding sign of ulcerative colitis that responded to treatment with methylprednisolone and infliximab.
Topics: Colitis, Ulcerative; Humans; Pyoderma Gangrenosum
PubMed: 35339135
DOI: No ID Found -
The Pan African Medical Journal 2020Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary... (Observational Study)
Observational Study
Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary study's aim was to examine all cases of PG observed in our department over a 6-year period in order to describe the relevant characteristics and outcome under therapy. Fourteen patients were included (5 women, 9 men). The average age of our patients was 40,15 years. The classical, ulcerative form was found in 10 cases (71.42%), the pustular form in 4 cases (27.57%) and PG was multifocal in 4 cases. The PG was located preferentially to the lower limbs. Histological examination was realized in all patients and objectified inflammatory infiltrate composed of polymorphonuclear neutrophils in all cases with vasculitis in 4 cases. Six patients (42.85%) had associated disease at diagnosis of PG, including inflammatory bowel disease in two cases (14.28%), a blood disease in 2 cases (14.28%), lymph node tuberculosis and inflammatory arthritis in 1 case (7%). The most frequent first-line treatments were oral corticosteroids (7 cases) and other treatments used were colchicine in 2 cases, topical corticosteroids in 3 cases with good clinical evolution. Our study confirms that PG is a rare disease, associated in almost half of cases with systemic disease already present at diagnosis; in our Moroccan background, it is most often inflammatory bowel disease, hematological or solid cancer and tuberculosis.
Topics: Adolescent; Adrenal Cortex Hormones; Adult; Aged; Child; Colchicine; Female; Humans; Male; Middle Aged; Prospective Studies; Pyoderma Gangrenosum; Treatment Outcome; Young Adult
PubMed: 32733629
DOI: 10.11604/pamj.2020.36.59.12329 -
Postepy Higieny I Medycyny... Mar 2016Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG). The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment.
METHODS
Systematic review of the literature in PubMed using the search terms "Wegener granulomatosis AND Pyoderma gangrenosum" and case report.
RESULTS
The finding of 22 reports in the literature (PubMed) suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide.
DISCUSSION
The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.
Topics: Face; Female; Granulomatosis with Polyangiitis; Humans; Neck; Necrosis; Neutrophils; Pyoderma Gangrenosum; Skin Ulcer; Young Adult
PubMed: 27117096
DOI: 10.5604/17322693.1197372 -
Head and Neck Pathology Dec 2017Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The... (Review)
Review
Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The condition is characterized by the appearance of one or more necrotic ulcers with a ragged undermined violaceous border and surrounding erythema. Lesions are often initiated by minor trauma. The condition can affect any anatomical site, however the head and neck are rarely involved. Although the oral cavity is subject to recurrent minor trauma through everyday activities such as mastication and oral hygiene, as well as during dental treatment, oral lesions appear to be extremely rare. In an effort to provide a detailed explanation of the oral manifestations of PG, a systematic search was conducted using medical databases. A total of 20 cases of PG with oral involvement were reported in the English and French literature. The objectives of this article are to present the pertinent diagnostic criteria and to discuss the differential diagnosis and therapeutic modalities.
Topics: Diagnosis, Differential; Humans; Oral Ulcer; Pyoderma Gangrenosum
PubMed: 28275955
DOI: 10.1007/s12105-017-0804-3 -
Deutsches Arzteblatt International Sep 2020
Topics: Aged; Biopsy; Chronic Disease; Humans; Male; Pyoderma Gangrenosum
PubMed: 33263537
DOI: 10.3238/arztebl.2020.0614a -
Indian Journal of Dermatology,... 2023Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and... (Review)
Review
BACKGROUND
Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and prognosis of pyoderma gangrenosum.
AIMS
We aimed to document clinicopathologic and prognostic data of the patients with pyoderma gangrenosum.
METHODS
In this retrospective observational study, we reviewed case records of patients diagnosed with pyoderma gangrenosum between 1999-2019.
RESULTS
Fifty-three patients were identified by reviewing medical records for skin biopsy; of these, 37 were men and 16 were women. Mean age at onset was 43.3 ± 18.5 years. The most frequently affected area was the lower extremities (60.4%), followed by the head and neck (17.0%). The most common subtype was ulcerative (47.2%), followed by bullous (22.6%). 30 cases had underlying diseases and the most common were malignancy (24.5%), followed by inflammatory bowel diseases (18.9%). The proportion of cases with history of trauma were significantly higher in post-operative type (100%) as compared to the bullous type (8.3%). Histologic features of granulation tissue were frequently found in post-operative type (66.7%) and bullous type (58.3%). Granulomas were predominantly found in bullous type (58.3%). Age <60 years appeared to be significantly associated with multiple lesions. Partial-to-complete remission was observed in 40 cases (75.5%). Nine (17.0%) cases experienced recurrence with a median progression-free period of six months (interquartile range of 3.0-9.0 months). Cases with underlying hematologic disorders and the bullous subtype were significantly associated with early recurrence.
LIMITATIONS
This study was a single-centre study with a retrospective design.
CONCLUSION
Pyoderma gangrenosum appears to have ethnic differences. Underlying haematologic disorders and bullous subtype have a worse prognosis. However, the type of histopathology did not correlate with the clinical outcome of pyoderma gangrenosum.
Topics: Male; Humans; Female; Young Adult; Adult; Middle Aged; Pyoderma Gangrenosum; Retrospective Studies; Inflammatory Bowel Diseases; Prognosis; Republic of Korea; Observational Studies as Topic
PubMed: 35389018
DOI: 10.25259/IJDVL_968_20 -
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