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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 -
Metabolites Jun 2023Bowel-associated arthritis-dermatosis syndrome (BADAS) is a rare neutrophilic dermatosis that was first described in 1971 in patients who underwent bypass surgery for... (Review)
Review
Bowel-associated arthritis-dermatosis syndrome (BADAS) is a rare neutrophilic dermatosis that was first described in 1971 in patients who underwent bypass surgery for obesity. Over the years, the number of reported cases associated with medical gastroenterological conditions, particularly inflammatory bowel disease (IBD), has progressively increased. To date, there are no systematic reviews in the literature on BADAS. The design of an a priori protocol was based on PRISMA guidelines, and a search of PubMed and Scopus databases was conducted for articles published between 1971 and 2023 related to the topic. Fifty-one articles including 113 patients with BADAS were analyzed in this systematic review. Bariatric surgery and IBD were the most frequently reported causes of BADAS, accounting for 63.7% and 24.7% of all cases, respectively. A total of 85% of cases displayed the typical dermatological presentation, including urticarial maculopapular lesions centered by a vesicopustule, with the majority of lesions located on the upper limbs (73.5%). Polyarthralgia or localized arthritis were always present. Atypical presentations included cellulitis-like, erythema-nodosum-like, Sweet-syndrome-like and pyoderma-gangrenosum-like manifestations. Gastrointestinal symptoms were frequently observed in IBD-related cases (67.9%). The histopathology showed a neutrophilic infiltrate (96.6%). The most commonly used treatment regimens consisted of systemic corticosteroids, metronidazole and tetracyclines, either alone or in combination. A relapsing-remitting course was observed in 52.1% of patients. In conclusion, BADAS is a neutrophilic dermatosis that presents with a wide variety of cutaneous manifestations, both typical and atypical. Gastrointestinal symptoms are frequently observed, particularly in cases related to IBD. The histopathology is clear but not specific compared with other neutrophilic dermatoses. The diagnosis can be challenging, but the relapsing-remitting course and the strong association with polyarthralgia and gastrointestinal disease can aid in the diagnosis.
PubMed: 37512497
DOI: 10.3390/metabo13070790 -
Pharmaceutics Jan 2022(1) Background: Colchicine is a natural alkaloid with anti-inflammatory properties used to treat various disorders, including some skin diseases. This paper aims to... (Review)
Review
(1) Background: Colchicine is a natural alkaloid with anti-inflammatory properties used to treat various disorders, including some skin diseases. This paper aims to incorporate all the available studies proposing colchicine as a treatment alternative in the management of cutaneous conditions. (2) Methods: In this systematic review, the available articles present in various databases (PubMed, Scopus-Embase, and Web of Science), proposing colchicine as a treatment for cutaneous pathological conditions, have been selected. Exclusion criteria included a non-English language and non-human studies. (3) Results: Ninety-six studies were included. Most of them were case reports and case series studies describing colchicine as single therapy, or in combination with other drugs. Hidradenitis suppurativa, pyoderma gangrenosum, erythema nodosum, erythema induratum, storage diseases, perforating dermatosis, bullous diseases, psoriasis, vasculitis, acne, urticaria, stomatitis, actinic keratosis, and pustular dermatosis were the main diseases discussed in literature. Although the therapeutic outcomes were variable, most of the studies reported, on average, good clinical results (4) Conclusions: Colchicine could be, as a single therapy or in combination with other drugs, a possible treatment to manage several skin diseases.
PubMed: 35214027
DOI: 10.3390/pharmaceutics14020294 -
Wound Repair and Regeneration :... Mar 2021Chronic painful ulcers caused by pyoderma gangrenosum (PG) and cutaneous vasculitis remain to be a therapeutic challenge. Skin grafts have been used with success in... (Review)
Review
Chronic painful ulcers caused by pyoderma gangrenosum (PG) and cutaneous vasculitis remain to be a therapeutic challenge. Skin grafts have been used with success in selected cases but are generally avoided due to the fear of pathergy. The aim of this study was to investigate the safety and efficacy of skin grafting in the treatment of primary vasculitic ulcer (PVU) and PG. MEDLINE, Embase, the Cochrane Library, Clinicaltrial.gov, and WHO International Clinical Trials Registry Platform (ICTRP) were searched from inception to March 2020. A search for grey literature was conducted in May 2020. We included studies assessing the efficacy and safety of skin grafting in the treatment of PG and PVU. Studies were only included if skin grafting was performed after establishment of PG or PVU diagnosis. A total of 721 articles was identified through the database search of which 92 were included in this study. Ten articles were identified by handsearching the reference list of included studies. Finally, 102 articles describing 212 wounds in 153 patients were included. Complete healing was found in 75.5% of the wounds. The average time to complete was 10.8 weeks (95% CI 6.1-15.6). The mean donor site healing time was 1.9 weeks (95% CI 0.52-3.20). Pathergy was reported in 8 (5.2%) patients. One patient had severe infection related to skin grafting. A statistically significant difference in the number of patients receiving preoperative (P = .0079) and postoperative (P = .002) immunosuppressive therapy was found between the groups with complete healing/reduction and no improvement/aggravation. This systematic review finds the current evidence on efficacy and safety of skin grafting in treatment of PG and PVU to be promising but limited to the size and lack of studies superior to case reports and case series. Future placebo-controlled trials are required to draw a stronger conclusion.
Topics: Humans; Pyoderma Gangrenosum; Skin Transplantation; Ulcer; Vasculitis; Wound Healing
PubMed: 33377584
DOI: 10.1111/wrr.12882 -
Journal of the American Academy of... Jun 2018Peristomal pyoderma gangrenosum (PPG) is an uncommon subtype of pyoderma gangrenosum. PPG is a challenging condition to diagnose and treat; no evidence-based guidelines...
BACKGROUND
Peristomal pyoderma gangrenosum (PPG) is an uncommon subtype of pyoderma gangrenosum. PPG is a challenging condition to diagnose and treat; no evidence-based guidelines exist.
OBJECTIVE
We sought to identify important clinical features of PPG and effective treatments available for its management.
METHODS
A systematic literature review of PPG was performed using PubMed, Medline, and Embase databases.
RESULTS
We describe 335 patients with PPG from 79 studies. Clinical features include a painful, rapidly progressing ulcer with undermined, violaceous borders with a history of ostomy leakage and local skin irritation or trauma. Systemic steroids are first-line therapy; infliximab and adalimumab provide concomitant control of active inflammatory bowel disease. Combination local and systemic therapy was commonly used. Wound dressings, vehicle selection, and appropriate ostomy devices to minimize leakage, irritation, and pressure-induced ischemia can improve healing. Distinct from classic ulcerative pyoderma gangrenosum, surgical approaches, such as stoma closure and resection of active inflammatory bowel disease, have an effective role in PPG management.
LIMITATIONS
PPG is a rare disease lacking randomized trials or diagnostic guidelines. Treatment duration and follow-up time among studies are variable.
CONCLUSIONS
Key clinical characteristics of PPG are highlighted. Several treatments, including a more prominent role for surgical intervention, may be effective for PPG treatment.
Topics: Adrenal Cortex Hormones; Anti-Bacterial Agents; Biological Products; Colitis, Ulcerative; Crohn Disease; Disease Management; Drug Therapy, Combination; Enterostomy; Female; Humans; Male; Postoperative Complications; Prognosis; Pyoderma Gangrenosum; Reoperation; Severity of Illness Index; Skin Care; Surgical Stomas
PubMed: 29288099
DOI: 10.1016/j.jaad.2017.12.049 -
The British Journal of Dermatology Nov 2020Pyoderma gangrenosum (PG) is a rare autoinflammatory skin condition that causes tissue destruction and subsequent painful ulcers. To date, there are no core domains or... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare autoinflammatory skin condition that causes tissue destruction and subsequent painful ulcers. To date, there are no core domains or instruments for assessing PG severity in clinical trials, and current treatment paradigms rely on outcome measurements that have not been well characterized in the literature.
OBJECTIVES
To perform two systematic reviews that (i) identify the outcome measurement instruments used in PG clinical trials and their corresponding domains and (ii) identify any associated validation studies and evaluate their measurement properties and methodological quality.
METHODS
We systematically searched the MEDLINE and Embase databases for PG outcome measurement instruments. We also systematically searched for PG instrument validation studies. We evaluated the measurement properties and methodological quality of validation studies using the 2018 COSMIN Risk of Bias checklist.
RESULTS
In total, seven clinical trials were included. These studies utilized a total of 20 different instruments, including 11 physician-reported instruments, eight patient-reported instruments and one composite instrument. Among these, 85% of the instruments lacked any validation data. Of the remaining three validated instruments (speed of healing, physician global assessment and resolution of inflammation), methodological quality was not available for half of the COSMIN categories.
CONCLUSIONS
We identified 17 non-validated outcome measurement instruments used in PG clinical trials. We conclude that PG validation studies are required for existing instruments, and new instruments need to be developed to inform the consensus process for the development of a core outcome set for PG. What is already known about this topic? Pyoderma gangrenosum (PG) is a rare autoinflammatory skin condition that has been characterized by multiple outcome measurement instruments in clinical trials. However, there is no consensus on the most validated and appropriate outcome measurement instruments. What does this study add? This study identifies and evaluates 20 unique outcome measurement instruments for PG in the literature. Of these 20, 17 lack any instrument validation data, highlighting the need for future studies. What are the clinical implications of this work? Despite the current use of several outcome measurement instruments, future studies should explore the validation surrounding these instruments, as no instruments can currently be recommended.
Topics: Checklist; Consensus; Databases, Factual; Humans; Pyoderma Gangrenosum
PubMed: 32159849
DOI: 10.1111/bjd.19027 -
Journal of Lower Genital Tract Disease Jul 2019The aim of the study was to review uncommon foreskin dermatopathology conditions clinically and pathologically.
OBJECTIVES
The aim of the study was to review uncommon foreskin dermatopathology conditions clinically and pathologically.
METHODS
A database search of PubMed and Google Scholar were extracted between March 1, 2009, and March 1, 2019, using the search terms "foreskin," "prepuce," "penis," "pathology," "dermatology," and "rare." The search was limited to "humans" and "dermatopathology." Full article texts were reviewed. Reference lists were screened for additional articles. Patient details (diagnosis, dermatopathology, treatment, and follow-up if available) were extracted. We excluded articles written in the non-English language, unusual variants of common conditions, and cases of common dermatologic conditions.
RESULTS
A list of 369 articles was identified and another screening identified 30 articles for rare foreskin pathologies. Those are divided into categories based on the following etiologies: (a) benign, including congenital (e.g., aposthia), infectious (graft versus host disease and histoplasma), autoimmune (Crohn's disease and pyoderma gangrenosum), and benign neoplasms (neurofibroma, apocrine hidrocystoma, verruciform xanthoma, porokeratosis, penile cutaneous horn, localized amyloidosis) and (b) malignancies, including primary (myeloid sarcoma, basal cell carcinoma, Kaposi's sarcoma, mucosal-associated lymphoid tissue lymphoma), and metastasis.
CONCLUSIONS
We reviewed and discussed unusual benign and malignant dermatopathology conditions that can affect the foreskin.
Topics: Adult; Aged; Autoimmune Diseases; Child; Child, Preschool; Dermatitis; Foreskin; Humans; Male; Middle Aged; Neoplasms; Penile Neoplasms
PubMed: 31149956
DOI: 10.1097/LGT.0000000000000478 -
Digestive Diseases and Sciences Sep 2020Pyoderma gangrenosum (PG) is an uncommon but severe extra-intestinal manifestation (EIM) of inflammatory bowel disease (IBD). The incidence and risk factors for PG are... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Pyoderma gangrenosum (PG) is an uncommon but severe extra-intestinal manifestation (EIM) of inflammatory bowel disease (IBD). The incidence and risk factors for PG are disputed.
AIMS
To assess the incidence of PG and identify factors associated with PG in IBD patients.
METHODS
A search of electronic databases (Ovid and PubMed) was conducted between 1966 and 2019. Studies that calculated the incidence of PG in IBD patient cohorts were included. Patient demographics, IBD subtype, and EIM presence were recorded. A review of our institutional database of 1057 IBD patients was conducted. A multivariate regression model and meta-analysis were conducted to identify risk factors for PG. A random effects model was used to combine the data of included studies.
RESULTS
Fourteen studies were included in addition to 1057 IBD patients and 26 PG cases from the Louisville cohort. In total, there were 379 cases of PG in the cumulative cohort of 61,695 IBD patients. The PG incidence in individual studies ranged from 0.4 to 2.6%. In the institutional cohort, ocular EIMs and a permanent stoma were significant risk factors for PG. In the meta-analysis, PG was associated with female gender (RR = 1.328, 95% CI 1.161-1.520), Crohn's disease (RR = 1.193, 95% CI 1.001-1.422), erythema nodosum (RR = 9.281, 95% CI 6.081-14.164), and ocular EIM (RR = 4.55, 95% CI 3.04-6.81). There was study heterogeneity when assessing IBD subtype, ocular, and joint EIMs.
CONCLUSIONS
There are conflicting data on the incidence and risk factors for PG. This meta-analysis confirms an association between PG and female gender, Crohn's disease, erythema nodosum, and ocular EIM that have been described in smaller studies.
Topics: Adult; Aged; Colitis, Ulcerative; Crohn Disease; Female; Humans; Incidence; Male; Middle Aged; Pyoderma Gangrenosum; Risk Assessment; Risk Factors; Sex Factors
PubMed: 31925675
DOI: 10.1007/s10620-019-05999-4 -
Journal of Plastic, Reconstructive &... Mar 2015Post-surgical pyoderma gangrenosum (PSPG) presents as a rapidly expanding cutaneous ulcer at a site of surgery with potentially devastating consequences. We... (Review)
Review
BACKGROUND
Post-surgical pyoderma gangrenosum (PSPG) presents as a rapidly expanding cutaneous ulcer at a site of surgery with potentially devastating consequences. We systematically reviewed the English and foreign language literature to identify risk factors for PSPG and propose a management strategy.
METHODS
A systematic review was completed in PubMed, Medline, Embase, and Cochrane Database for all published reports of PSPG from January 1946 to June 2013. We manually examined bibliographies for relevant references and used Google Translate for articles in foreign languages, including Italian, Japanese, German, Dutch, Turkish, Spanish, Chinese, Dutch, Russian, Portuguese, and Czech.
RESULTS
We identified 220 cases of PSPG (mean age 52.8 years, range 5-85 years). Thirty-seven patients (16.8%) had a history of pyoderma gangrenosum, nineteen (8.6%) had a hematologic disorder such as leukemia or lymphoma, thirteen (5.9%) had inflammatory bowel disease, and eight (3.6%) had rheumatoid arthritis. PSPG occurred most commonly after breast (25%), cardiothoracic (14%), abdominal (14%), and obstetric (13%) surgeries. The most common breast procedures were bilateral reduction mammoplasty (45%), breast reconstruction (25%), and lumpectomy or mastectomy (11%). Signs of wound complication occurred on average 7.0 days after surgery. Nineteen patients (8.6%) at risk for PSPG received perioperative corticosteroids during skin grafting or later surgeries with a favorable outcome.
CONCLUSIONS
Patients with a history of pyoderma gangrenosum, rheumatoid arthritis, inflammatory bowel disease, or hematologic malignancy who are undergoing breast, cardiothoracic, or abdominal surgeries should be carefully observed for post-operative ulceration at incision sites. Debridement should not be performed before dermatologic consultation to assess for PSPG. Patients at risk of PSPG undergoing breast surgery may benefit from perioperative prednisone to prevent PSPG which can lead to destructive wound enlargement and significant scarring.
Topics: Humans; Postoperative Complications; Pyoderma Gangrenosum; Risk Factors; Surgical Wound Infection
PubMed: 25589459
DOI: 10.1016/j.bjps.2014.12.036 -
Medicina (Kaunas, Lithuania) Aug 2020Background and Objectives Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated...
UNLABELLED
Background and Objectives Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated with numerous extraintestinal manifestations, making its management a real challenge for the pediatric gastroenterologist. Dermatological manifestations in IBD are either specific, related to the disease activity or treatment-associated, or non-specific. This literature review aims to identify and report the dermatological manifestations of IBD in children, the correlation between their appearance and the demographical characteristics, the relationship between these lesions and disease activity, and to highlight the impact of dermatological manifestations on an IBD treatment regime.
MATERIALS AND METHODS
A systemic literature review was performed, investigating articles and case reports on dermatological manifestations in children with IBD starting from 2005. A total of 159 potentially suitable articles were identified and after the exclusion process, 75 articles were selected.
RESULTS
The most common dermatological manifestations reported in pediatric IBD are erythema nodosum and pyoderma gangrenosum. More rare cases of metastatic Crohn's disease, epidermolysis bullosa acquisita, small-vessel vasculitis, necrotizing vasculitis, leukocytoclastic vasculitis, cutaneous polyarteritis nodosa, and Sweet's syndrome have been reported. Oral manifestations of IBD are divided into specific (tag-like lesions, mucogingivitis, lip swelling with vertical fissures, aphthous stomatitis, and pyostomatitis vegetans) and non-specific. IBD treatment may present with side effects involving the skin and mucosa. Anti-tumor necrosis factor agents have been linked to opportunistic skin infections, psoriasiform lesions, and a potentially increased risk for skin cancer. Cutaneous manifestations such as acrodermatitis enteropathica, purpuric lesions, and angular cheilitis may appear secondary to malnutrition and/or malabsorption.
CONCLUSIONS
The correct diagnosis of dermatological manifestations in pediatric IBD is of paramount importance because of their impact on disease activity, treatment options, and a patient's psychological status.
Topics: Biological Factors; Child; Colitis, Ulcerative; Crohn Disease; Humans; Immunosuppressive Agents; Malabsorption Syndromes; Receptors, Tumor Necrosis Factor; Skin Diseases
PubMed: 32842528
DOI: 10.3390/medicina56090425