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European Review For Medical and... Jul 2022Pyoderma Gangrenosum (PG) is an immune-mediated neutrophilic dermatosis, characterized by large painful ulcers occurring in various body segments. It can be associated... (Review)
Review
OBJECTIVE
Pyoderma Gangrenosum (PG) is an immune-mediated neutrophilic dermatosis, characterized by large painful ulcers occurring in various body segments. It can be associated to Inflammatory Bowel Disease (IBD) including both Ulcerative Colitis and Crohn Disease. Prompt and effective management is fundamental, due to its high morbidity and mortality rates. By presenting our clinical experience, we aimed at showing the efficacy of a combined therapeutic approach, in which the best of every specialty cooperates managing this hazardous disease.
PATIENTS AND METHODS
We report on two patients attending our outpatient clinic with ulcerative skin lesions at the level of the back. Patient 1 suffered from Crohn disease and Patient 2 presented a positive history of abdominal pain, diarrhea with mucus and blood in the stool. Histological exam was performed with final diagnosis of PG associated with IBD. A Literature review was carried out in order to highlight the role of combined clinical-surgical management of PG in adult patients with IBD.
RESULTS
Complete resolution of the lesions was achieved in 4 months and 3 months for each patient respectively without relapse. PubMed was searched from 2000 to 2020 with the following keywords: (Pyoderma) AND/OR (Pyoderma Gangrenosum) AND (Inflammatory Bowel Disease) AND/OR (Ulcerative Colitis) AND/OR (Crohn Disease) AND (Management). Seven papers were included (4 case reports, 2 case series, 1 comprehensive review) and reviewed using a descriptive checklist.
CONCLUSIONS
PG should be treated by dedicated multidisciplinary teams, in which every specialist plays a crucial role from the diagnosis to the treatment and up to the long-term follow-up.
Topics: Adult; Chronic Disease; Colitis, Ulcerative; Crohn Disease; Humans; Inflammatory Bowel Diseases; Pyoderma Gangrenosum; Recurrence
PubMed: 35916817
DOI: 10.26355/eurrev_202207_29308 -
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 -
The Journal of Investigative Dermatology Apr 2022
Topics: Gene Expression; Humans; Pyoderma Gangrenosum
PubMed: 34536481
DOI: 10.1016/j.jid.2021.08.431 -
Advances in Wound Care Jul 2020Pyoderma gangrenosum (PG) is a rare debilitating autoinflammatory ulcerative skin disease. No gold standard has been established for the treatment of PG. The role of... (Review)
Review
Surgical Treatment of Pyoderma Gangrenosum with Negative Pressure Wound Therapy and Skin Grafting, Including Xenografts: Personal Experience and Comprehensive Review on 161 Cases.
Pyoderma gangrenosum (PG) is a rare debilitating autoinflammatory ulcerative skin disease. No gold standard has been established for the treatment of PG. The role of surgical interventions and negative pressure wound therapy (NPWT) was discussed controversially until recently as these procedures might pose a trigger to further aggravate the condition. Recent advances confirm the paradigm change that a surgical approach of PG with split thickness skin grafting (STSG) secured by NPWT is a safe and valuable treatment if performed under adequate immunosuppression. We elaborate this on the hand of a broad literature search retrieving 101 relevant articles describing 138 patients complemented with our personal experience on 23 patients, including 2 patients treated with a porcine xenodressing. A wide range of surgical approaches have been reported, including xenografts. Treatment was finally successful in 86%, including the xenotransplant cases. Ten percent improved and failures were mainly reported without immunosuppression. Despite halting the inflammatory process, NPWT alone, without skin grafting, does not much accelerate healing time. The best surgical approach appears to be STSG fixed with NPWT as this leads to higher skin graft take. There remains the problem of the chronic nature of PG and the recurrence after tapering of immunosuppression or trauma; therefore, a sustained immunosuppressive treatment is suggested. While surgical treatment is supported by the published data, the exact immunosuppression is still evolving. Due to deeper insights into pathogenesis and growing clinical reports, a broader utilization of biologic treatments and a shift from tumor necrosis factor (TNF)-alpha to interleukin (IL)-12/23 or IL-23 antibodies alone are predictable, as IL-12/23 antibodies show good clinical responses with fewer side effects. The positive results with porcine xenodressings might be due to immunological effects of the xenomaterial; they appear promising, but are preliminary and should be confirmed in a larger patient collective.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Animals; Child; Female; Heterografts; Humans; Immunosuppressive Agents; Male; Middle Aged; Negative-Pressure Wound Therapy; Pyoderma Gangrenosum; Recurrence; Secondary Prevention; Skin; Skin Transplantation; Swine; Transplantation, Heterologous; Treatment Outcome; Wound Healing; Young Adult
PubMed: 32320362
DOI: 10.1089/wound.2020.1160 -
Frontiers in Immunology 2021This study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China...
OBJECTIVES
This study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China and provide an up-to-date literature review.
METHODS
The clinical data and genotype of three Chinese Han patients were carefully documented and studied. We also conducted a systematic literature review on PAPA syndrome.
RESULTS
A total of three patients were diagnosed with PAPA syndrome at our center from 2018 to 2020. Arthritis was observed in all three patients, while pyoderma gangrenosum (PG) was found in two patients and acne in one patient. Other manifestations included pathergy reaction, intermittent fever, oral ulcer, keratitis, proteinuria, and hematuria. The A230T mutation was identified in two patients, and a novel Y119C variation was revealed in a sporadic patient. A total of 76 patients with PAPA syndrome reported in 29 articles were included in our literature review. The classical triad of arthritis, PG, and acne was visible in only 16 (25.4%) patients, while 24 (38.1%) exhibited only one major symptom. Skin lesions were more commonly seen in patients with adult-onset disease than those with childhood-onset disease (100 . 83%), whereas arthritis was less common (50 . 98.1%). Steroid and/or biological agents were effective in most patients.
CONCLUSIONS
The rarity and phenotypic heterogeneity associated with PAPA syndrome make the diagnosis a huge challenge to physicians, especially in adult patients. A significant portion of patients did not exhibit the full spectrum of the classical triad. Accordingly, gene testing is critically helpful for diagnosis.
Topics: Acne Vulgaris; Adaptor Proteins, Signal Transducing; Adult; Arthritis, Infectious; Biological Products; Cytoskeletal Proteins; DNA Mutational Analysis; Female; Genetic Predisposition to Disease; Humans; Male; Middle Aged; Mutation; Phenotype; Predictive Value of Tests; Pyoderma Gangrenosum; Steroids; Treatment Outcome
PubMed: 34745107
DOI: 10.3389/fimmu.2021.735851 -
The Tokai Journal of Experimental and... Sep 2017Pyoderma gangrenosum is an intractable disease of unknown cause involving recurrent ulcerative lesions on the skin, and may accompany ulcerative colitis, rheumatoid...
Pyoderma gangrenosum is an intractable disease of unknown cause involving recurrent ulcerative lesions on the skin, and may accompany ulcerative colitis, rheumatoid arthritis, leukemia, systemic lupus erythematosus, and other conditions. Here, we report a rare case of pyoderma gangrenosum in the thoracic abdomen following post-mastectomy reconstructive surgery. A 39-year-old presented at the hospital with a complaint of left papilla erosion. Skin biopsy at the site revealed invasive skin cancer, with Paget-like progression in the cancerous nipple and suspected malignancy of skin appendages. After partial mastectomy including the areola, invasive ductal breast carcinoma was diagnosed. The patient underwent a subsequent full mastectomy with simultaneous sentinel lymph node biopsy and primary breast reconstructive surgery using a rectus abdominis myocutaneous flap. Two weeks post-surgery, healing of the abdominal surgical wound was found to be delayed, and suture abscess was suspected. Despite localized treatment, an ulcerative lesion developed in the thoracic region, and pyoderma gangrenosum was diagnosed following skin biopsy. After the introduction of steroid pulse therapy, no progression of the lesion was observed. This report describes the disease characteristics, diagnosis, and treatment of post-surgical pyoderma gangrenosum and discusses the case in the context of previous literature.
Topics: Adult; Breast Neoplasms; Carcinoma, Ductal, Breast; Female; Humans; Mammaplasty; Mastectomy; Postoperative Complications; Prednisolone; Pulse Therapy, Drug; Pyoderma Gangrenosum; Rectus Abdominis; Surgical Flaps; Treatment Outcome
PubMed: 28871582
DOI: No ID Found -
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 -
Ophthalmic Plastic and Reconstructive...A 72-year-old woman with a history of chronic cocaine use presented 9 months after a dog bite with a large facial ulceration and absent sinonasal structures. Biopsies... (Review)
Review
A 72-year-old woman with a history of chronic cocaine use presented 9 months after a dog bite with a large facial ulceration and absent sinonasal structures. Biopsies were negative for infectious, vasculitic, or neoplastic pathologies. The patient was lost to follow up for 15 months and returned with a significantly larger lesion despite abstinence from cocaine. Additional inflammatory and infectious workup was negative. Intravenous steroids were administered with clinical improvement. Therefore, she was diagnosed with pyoderma gangrenosum and cocaine-induced midline destructive lesion due to cocaine/levamisole. Pyoderma gangrenosum is a rare dermatologic condition that uncommonly involves the eye and ocular adnexa. Diagnosis involves clinical examination, response to steroids, exclusion of infectious or autoimmune conditions, and identifying potential triggers including cocaine/levamisole. This report highlights a rare presentation of periorbital pyoderma gangrenosum causing cicatricial ectropion associated with concomitant cocaine-induced midline destructive lesion and reviews important aspects of clinical manifestations, diagnosis, and management of pyoderma gangrenosum and cocaine/levamisole autoimmune phenomenon.
Topics: Female; Animals; Dogs; Humans; Cocaine; Pyoderma Gangrenosum; Levamisole; Face; Skin Ulcer
PubMed: 36893063
DOI: 10.1097/IOP.0000000000002347 -
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 -
Wounds : a Compendium of Clinical... Sep 2017Pyoderma gangrenosum (PG) is a rare ulcerative condition that is diagnostically and therapeutically challenging, as debridement leads to ulcer deterioration (pathergy... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare ulcerative condition that is diagnostically and therapeutically challenging, as debridement leads to ulcer deterioration (pathergy phenomenon); immunosuppression is considered the gold standard therapy.
CASE REPORT
The authors present the case of a 42-year-old woman with PG and uncontrolled iatrogenic diabetes, secondary to a total pancreatectomy performed in another hospital in 1998 due to nesidioblastosis. In 2008, she was referred to the diabetic foot consultation at Centro Hospitalar Tondela-Viseu (Viseu, Portugal) due to an infected wound on the left leg thought to be related to a trauma from footwear, but the injury worsened despite treatment. Characteristics of the lesion led to the diagnosis of PG and treatment was adjusted. The patient was followed-up weekly at the diabetic foot consultation; at 2-months follow-up, the leg ulcer was closed but additional lesions emerged in multiple areas of her body. Therapy included local antiseptics, antibiotics, pain control, systemic corticosteroids, immunosuppressors, intravenous immunoglobulin, and surgical debridement. Due to her noncompliance, this proved to be a challenging case.
CONCLUSION
Although PG is rare, clinicians should suspect it in the presence of purplish wound edges with a necrotic center. Surgical debridement, while necessary in other ulcers, can worsen the condition and should only be applied to self-detachable necrotic plaques.
Topics: Adult; Debridement; Female; Humans; Immunosuppression Therapy; Leg Ulcer; Pyoderma Gangrenosum; Treatment Outcome
PubMed: 28933699
DOI: No ID Found