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The British Journal of Dermatology Feb 2018Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. Treatment regimens for refractory cases are nonstandardized. Intravenous immunoglobulin (IVIG) is an... (Meta-Analysis)
Meta-Analysis
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. Treatment regimens for refractory cases are nonstandardized. Intravenous immunoglobulin (IVIG) is an emerging treatment with reported success, but the efficacy of IVIG for PG is unknown. In this systematic review of cases and case series, we assessed the efficacy of IVIG for the treatment of PG, as observed at our institution and reported in the literature. A retrospective chart review at two tertiary care hospitals between 2000 and 2015, and literature searches in PubMed/MEDLINE, EMBASE and Web of Science from all years were conducted. In total, there were 49 patients, including 43 patients from 26 articles and six institutional cases. There was complete or partial response in 43 (88%) patients and complete response in 26 (53%) patients. The mean time to initial response to treatment and treatment length were 3·5 (SD 3·3) weeks and 5·9 (SD 7·8) months, respectively. On average, 2·6 treatments had been trialled before IVIG initiation. IVIG was administered with systemic steroids in 43 (88%) cases. Mild adverse events, especially nausea and headache, were reported in 12 (24·5%) patients. Our systematic review suggests a potential role for IVIG as adjuvant therapy for refractory PG. Prospective clinical trials testing the efficacy of IVIG for refractory PG are needed to validate these findings.
Topics: Adjuvants, Immunologic; Adolescent; Adult; Aged; Aged, 80 and over; Chronic Disease; Female; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Pyoderma Gangrenosum; Treatment Outcome; Young Adult
PubMed: 28742926
DOI: 10.1111/bjd.15850 -
Plastic and Reconstructive Surgery.... Apr 2017Pyoderma gangrenosum (PG) is a rare skin disorder of the neutrophilic dermatoses spectrum that can mimic wound infections in surgical patients. PG after breast surgery...
BACKGROUND
Pyoderma gangrenosum (PG) is a rare skin disorder of the neutrophilic dermatoses spectrum that can mimic wound infections in surgical patients. PG after breast surgery has been reported but in limited amounts in autologous breast reconstruction patients. We present the first case of PG after a delayed bilateral deep inferior epigastric perforator flap breast reconstruction in the setting of systemic disease along with a systematic review.
METHODS
PubMed, Ovid, and Web of Science were systematically searched to obtain cases of PG after autologous breast reconstruction. Sixty articles were identified but only 16 were relevant to this study.
RESULTS
Systemic disease was present in 2 patients (13%). Wound onset occurred typically 5 days after surgery. Fever and/or leukocytosis was observed in 10 patients (63%). Wound cultures were positive in 2 patients (13%). Donor-site wounds were present in 9 patients (56%). Bilateral breast wounds were present in 67% of bilateral cases. Debridement was performed in 10 cases (63%). Skin graft or substitute was performed in 6 cases (38%). Resection of autologous flap was performed in 3 cases (19%). All patients were treated with systemic steroids (81%) and/or immunosuppressive medications (50%). Complete wound healing occurred by 4 months on average.
CONCLUSION
If early ulcerative wounds develop at multiple sites after autologous breast reconstruction with worsening after debridement and antibiotic therapy, then PG should be considered. It is imperative that an early diagnosis and subsequent treatment with steroids and/or immunosuppressive medications be initiated so further surgical procedures, flap loss, and scarring can be minimized.
PubMed: 28507842
DOI: 10.1097/GOX.0000000000001239 -
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 -
International Journal of Dermatology May 2017Pyoderma gangrenosum (PG) is a sterile neutrophilic disorder that rarely affects children. Clinical, epidemiological, and therapeutic data on pediatric PG is poor as... (Review)
Review
Pyoderma gangrenosum (PG) is a sterile neutrophilic disorder that rarely affects children. Clinical, epidemiological, and therapeutic data on pediatric PG is poor as there are many newly reported associated diseases and drugs. This paper aims to review all recent available data on pediatric PG. A systematic review of the literature was conducted using Embase, Medline, and Cochrane databases. A total of 132 articles were included in the review. The most commonly reported underlying diseases in pediatric PG are inflammatory bowel diseases followed by hematologic disorders, vasculitis, immune deficiencies and Pyogenic Arthritis, Pyoderma gangrenosum and Acne (PAPA) syndrome. More than half of the cases occur with no underlying disease. The most frequently reported clinical presentation is multiple disseminated ulcers. Treatment should be tailored according to the underlying etiology. It includes systemic steroids, corticosteroid sparing agents such as dapsone and cyclosporine, and TNF-alpha inhibitors such as adalimumab and infliximab. Response to treatment is high with cure rates reaching 90%. A high index of suspicion and a thorough workup are mandatory in the management of pediatric PG.
Topics: Acne Vulgaris; Adolescent; Arthritis, Infectious; Child; Child, Preschool; Hematologic Diseases; Humans; Immunologic Deficiency Syndromes; Infant; Infant, Newborn; Inflammatory Bowel Diseases; Pyoderma Gangrenosum; Vasculitis
PubMed: 28233293
DOI: 10.1111/ijd.13584 -
Journal of the American Academy of... Mar 2017Pyoderma gangrenosum (PG) is a rare, ulcerative cutaneous disorder. Ophthalmic involvement in PG is atypical, but can have devastating consequences. (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare, ulcerative cutaneous disorder. Ophthalmic involvement in PG is atypical, but can have devastating consequences.
OBJECTIVE
We sought to characterize ocular PG to allow for earlier diagnosis and therapy. To our knowledge, this is the first systematic review summarizing this clinical variant.
METHODS
A systematic review was conducted using PubMed and Web of Science. Data were extracted and studies were qualitatively assessed and analyzed.
RESULTS
We identified all 34 cases of PG involving the eye and periorbital area, and categorized them into 4 different subtypes. Common presenting signs include ulceration, peripheral ulcerative keratitis, and decreased visual acuity. Although it is often difficult to biopsy ocular PG, histologic features are nonspecific. Combined therapy using corticosteroids and further surgical reconstruction as needed is the mainstay of treatment. Cases of the eye/orbit in particular should be treated aggressively, as these are more likely to relapse compared with cases of the periorbital area.
LIMITATIONS
Use of case reports, paucity of ocular PG cases, and heterogeneity of studies are limitations.
CONCLUSION
PG should be considered in the differential diagnosis of ulceration of ocular/periocular tissues. An aggressive, early, multimodal treatment strategy should be used to prevent relapse, especially in cases of the eye/orbit.
Topics: Comorbidity; Diagnosis, Differential; Eye Diseases; Humans; Pyoderma Gangrenosum
PubMed: 27836332
DOI: 10.1016/j.jaad.2016.08.049 -
Clinical Gastroenterology and... Jan 2017This systematic review investigated the efficacy and the effectiveness of biologic drugs in extraintestinal manifestations (EIMs) in inflammatory bowel disease (IBD). (Review)
Review
BACKGROUND & AIMS
This systematic review investigated the efficacy and the effectiveness of biologic drugs in extraintestinal manifestations (EIMs) in inflammatory bowel disease (IBD).
METHODS
Literature search was conducted in PubMed, Embase, and Cochrane until October 2015. Main inclusion criteria were adults with IBD, use of a biologic drug, evolution of EIMs, interventional study, or non-interventional study.
RESULTS
Nine interventional studies (2 randomized controlled trials [N = 797], 7 open label trials [N = 1143], and 13 non-interventional studies [N = 914]) were included. Tumor necrosis factor (TNF) antagonists achieved complete response for pyoderma gangrenosum in 21%-25% of patients in interventional studies and in 92%-100% patients in non-interventional studies, with similar results for other cutaneous manifestations such as erythema nodosum or stomatitis. Adalimumab significantly reduced the prevalence of anemia vs placebo after 56 weeks in 1 randomized controlled trial. In 2 non-interventional studies, anti-TNF therapy improved anemia in the short-term (67%) and in the long-term (34%). Complete response after anti-TNF treatment was reported in interventional studies, including arthralgia (reduction in prevalence from 47.1% to 26.8% in the mid-term in 1 open label trial) and arthritis (reduction in prevalence from 8.7% to 2.1% and from 58% to 12.5% in 2 open label trials). Anti-TNFs were beneficial for a majority of patients with ocular manifestations. Infliximab was associated with improved outcomes in bone formation and bone mineral density.
CONCLUSIONS
Anti-TNFs appear to be effective alternatives for certain EIMs associated with IBD including musculoskeletal, cutaneous, and ocular manifestations, and some beneficial effect may be obtained in metabolic bone disease and on hematologic or vascular EIMs.
Topics: Biological Products; Humans; Immunologic Factors; Inflammatory Bowel Diseases; Treatment Outcome; Tumor Necrosis Factor-alpha
PubMed: 27392760
DOI: 10.1016/j.cgh.2016.06.025 -
Journal of the European Academy of... Feb 2017
Review
Topics: Adolescent; Adult; Female; Humans; Male; Middle Aged; Negative-Pressure Wound Therapy; Pyoderma Gangrenosum; Skin Transplantation; Young Adult
PubMed: 27225541
DOI: 10.1111/jdv.13727 -
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 -
PloS One 2015We conducted a comprehensive, systematic review of the global childhood population prevalence of impetigo and the broader condition pyoderma. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
We conducted a comprehensive, systematic review of the global childhood population prevalence of impetigo and the broader condition pyoderma.
METHODS
PubMed was systematically searched for impetigo or pyoderma studies published between January 1 1970 and September 30 2014. Two independent reviewers extracted data from each relevant article on the prevalence of impetigo.
FINDINGS
Sixty-six articles relating to 89 studies met our inclusion criteria. Based on population surveillance, 82 studies included data on 145,028 children assessed for pyoderma or impetigo. Median childhood prevalence was 12·3% (IQR 4·2-19·4%). Fifty-eight (65%) studies were from low or low-middle income countries, where median childhood prevalences were 8·4% (IQR 4·2-16·1%) and 14·5% (IQR 8·3-20·9%), respectively. However, the highest burden was seen in underprivileged children from marginalised communities of high-income countries; median prevalence 19·4%, (IQR 3·9-43·3%).
CONCLUSION
Based on data from studies published since 2000 from low and low-middle income countries, we estimate the global population of children suffering from impetigo at any one time to be in excess of 162 million, predominantly in tropical, resource-poor contexts. Impetigo is an under-recognised disease and in conjunction with scabies, comprises a major childhood dermatological condition with potential lifelong consequences if untreated.
Topics: Child; Child, Preschool; Humans; Impetigo; Prevalence; Pyoderma; Socioeconomic Factors
PubMed: 26317533
DOI: 10.1371/journal.pone.0136789 -
Aesthetic Plastic Surgery Aug 2015Pyoderma gangrenosum (PG) is a rare autoinflammatory neutrophilic ulcerative skin disease, often developing after a trauma or surgical wounds. In the literature there... (Review)
Review
INTRODUCTION
Pyoderma gangrenosum (PG) is a rare autoinflammatory neutrophilic ulcerative skin disease, often developing after a trauma or surgical wounds. In the literature there are several reports of post-surgical PG (PSPG) of the breast. The authors of this article experienced an impressive case of PSPG after an aesthetic breast augmentation mastopexy. PSPG is a rare but severe complication in this elective aesthetic surgical procedure.
METHOD
A systematic review of the literature was performed, focusing on PSPG after aesthetic breast surgery (augmentation mammoplasty/mastopexy). The online databases Pubmed, Medline, and Cochrane were used and additionally a Google© search was conducted. We compared the data obtained from a systematic literature review to an index case of PSPG after esthetic augmentation mammoplasty.
RESULTS
The literature search identified seven articles describing eight cases of PSPG after aesthetic breast surgery. In four of these cases augmentation mammoplasty had been carried out, in two cases mastopexy and in two cases augmentation mammoplasty and mastopexy (augmentation mastopexy). The patient we treated and describe in this paper underwent an augmentation mastopexy outside our clinic. Eight patients suffered from local disease, at the site of surgical wounds, one patient had disseminated disease. Leukocytosis was present in five cases (out of nine). Eight patients had received corticosteroid treatment, one patient refused such treatment. The duration of corticosteroid treatment was on average for 41 days (range 21-60 days). In all cases, the areola had been spared. Complete healing of PSPG was observed on average after 5 months (range 1.5 months-1 year).
DISCUSSION
PSPG of the breast after aesthetic breast surgery is rare, but every plastic surgeon should consider this possibility, especially if skin disease develops post-surgery, mimicking wound infection that does not respond to broad-spectrum antibiotic treatment.
CONCLUSION
Although the literature does not recommend this step, implant removal is recommended by the authors because bacterial wound infection normally cannot be ruled out definitely in the early stages of disease. Additional surgical intervention should be limited to the absolute necessary and performed only under adequate systemic immunosuppressive therapy.
Topics: Adult; Ambulatory Care Facilities; Female; Humans; Mammaplasty; Pyoderma Gangrenosum
PubMed: 26017179
DOI: 10.1007/s00266-015-0499-3