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Baco SJ, Mišić J, Peruničić V. Postoperative Pyoderma Gangrenosum (PPG) After Appendectomy: A Case Report. Cureus 2023; 15:e42016. [PMID: 37469578 PMCID: PMC10353335 DOI: 10.7759/cureus.42016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/17/2023] [Indexed: 07/21/2023] Open
Abstract
Pyoderma gangrenosum (PG) is a rare, poorly understood, non-infectious, autoimmune phenomenon. It is an inflammatory neutrophilic dermatosis characterized by hyperactivity of the skin and the development of papules and pustules that rapidly progress to painful ulcerations with a violaceous and necrotic border. Approximately three to six cases of PG per million of the population occur per year and in the case of postoperative pyoderma gangrenosum (PPG), it is only one to three cases per million operated people. We are presenting a 41-year-old patient with a clinical presentation of PPG, developed in the surgical site on the sixth postoperative day (POD 6) following open appendectomy for acute appendicitis. Initial treatment was for surgical site infection (SSI) with wound opening, regular dressings, and broad-spectrum antibiotics. Due to unresponsiveness to therapy and the unexpected postoperative course with the progression of skin lesions, we suspected PPG. Corticosteroid therapy was introduced in a shock dose, once daily intravenous (IV), with superb results and stopping the spread of the process after only two days. Considering the rarity of PPG, especially when it first occurs postoperatively, we believe that the image of skin changes with superficial spreading and characteristic violaceous ulcerations can be of crucial importance for early diagnosis. A multidisciplinary approach with a mandatory examination by a dermatologist is important in order to make an early diagnosis and prevent wrong treatment, with the potential worsening of the patient's condition. Atraumatic wound care and negative pressure wound therapy are recommended. Patients at risk should perioperatively receive corticosteroids and postoperatively be closely observed for the potential development of PPG. Debridement is not recommended, and surgical treatment and further tissue trauma are undesirable and even prohibited.
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Affiliation(s)
- Stanko J Baco
- Department of General Surgery, Public Health Institution Hospital "Dr Mladen Stojanovic", Prijedor, BIH
| | - Jovica Mišić
- Department of General Surgery, Saint Luke the Apostle Hospital, Doboj, BIH
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Ohmura SI, Homma Y, Hanai S, Otsuki Y, Miyamoto T. Successful switching treatment of adalimumab for refractory pyoderma gangrenosum in a patient with rheumatoid arthritis with prior use of tumour necrosis factor inhibitors: A case report and review of the literature. Mod Rheumatol Case Rep 2023; 7:9-13. [PMID: 35285489 DOI: 10.1093/mrcr/rxac023] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2022] [Revised: 03/02/2022] [Accepted: 03/10/2022] [Indexed: 01/07/2023]
Abstract
Pyoderma gangrenosum (PG) is a rare chronic skin disease characterised by painful skin ulcers. There are no treatment guidelines for PG, but systemic treatments including biologics are often used. Recently, adalimumab (ADA), a fully human monoclonal antibody against tumour necrosis factor, was approved for refractory PG treatment in Japan. Herein, we report a case of rheumatoid arthritis with refractory PG 2 months after orthopaedic surgery of the foot during treatment with low-dose etanercept and methotrexate. Although adding a moderate dose of glucocorticoid did not improve her PG, the patient showed a remarkable response after switching from etanercept to ADA in a higher dose than that used to treat rheumatoid arthritis. This higher dose of ADA may be effective for the treatment of refractory PG after the failure of other tumour necrosis factor inhibitors.
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Affiliation(s)
- Shin-Ichiro Ohmura
- Department of Rheumatology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Yoichiro Homma
- Department of General Internal Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Shiho Hanai
- Department of Dermatology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Yoshiro Otsuki
- Department of Pathology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Toshiaki Miyamoto
- Department of Rheumatology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
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3
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Magro CM, Mo JH, Kerns MJ. Leukocytoclastic vasculitis in association with linear epidermal basement membrane zone immunoglobulin deposition: Linear vasculitis. Clin Dermatol 2022; 40:639-650. [PMID: 35907580 DOI: 10.1016/j.clindermatol.2022.07.011] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Cutaneous leukocytoclastic vasculitis (LCV) has a distinctive clinical and light microscopic presentation; however, the etiologic basis of LCV is varied. Most cases are attributable to immune complex deposition within a vessel wall and represent an Arthus type III immune complex reaction. The prototypic immunoreactant profile is characterized by granular deposits of components of complement activation in concert with immunoglobulin within the cutaneous vasculature. We encountered nine patients with vasculitic and/or vesiculobullous clinical presentations exhibiting an LCV in association with an immunoreactant profile characterized by homogeneous linear deposits of immunoglobulin along the dermal epidermal junction in a fashion resembling an autoimmune vesiculobullous disease. Among the clinical presentations were palpable purpura, urticarial vasculitis, and vesiculobullous eruptions with supervening purpura. Two patients with Crohn disease presented with classic palpable purpura with biopsy-proven LCV, and direct immunofluorescence (DIF) studies demonstrated linear immunoglobulin G (IgG) with floor localization on the salt-split skin assay. Four patients with systemic lupus erythematosus (SLE) showed purpuric vesiculobullous lesions, with evidence of a neutrophilic interface dermatitis and LCV in three of the four. The remaining patient had urticarial nonbullous lesions showing small-vessel vasculitiswith a neutrophilic interface dermatitis. In all of the patients with SLE, DIF studies showed linear immunoglobulin deposits within the basement membrane zone (BMZ). These constellation of findings clinically, light microscopically, and by immunofluorescence were those of a vasculitic presentation of bullous systemic lupus erythematosus. Two patients had linear IgA disease, which was drug induced in one and paraneoplastic in the other, and the dominant morphology on biopsy in both cases was an LCV. One patient microscopically demonstrated drug-associated and eosinophilic enriched LCV with DIF studies showing striking linear deposits of IgG suggestive of bullous pemphigoid, which was consistent with a vasculitic presentation of drug-induced bullous pemphigoid. In all cases, typical granular vascular immunoglobulin and complement deposition compatible with immune complex mediated vasculitis was observed. It is likely that local immune complexes derived from BMZ antigen bound to antibody are pathogenically relevant. We propose the designation of linear vasculitis for this unique scenario of LCV and linear immunoglobulin epidermal BMZ staining, which in some cases represents a vasculitic presentation of conventional autoimmune vesiculobullous disease.
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Affiliation(s)
- Cynthia M Magro
- Division of Dermatopathology, Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA.
| | - Joshua H Mo
- Division of Dermatopathology, Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA
| | - Mary Jo Kerns
- Dermatologists of Southwest Ohio, Office 3555 Olentangy River Rd Suite 4000, 43214, Columbus, Ohio, USA
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Giraudo F, Miraglia E, Garbi ML, Yantorno M, Maradeo MR, Correa GJ, Tufare F. Prevalence of pyoderma gangrenosum and Sweet´s syndrome in inflammatory bowel disease at a tertiary healthcare center. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2020; 113:691-697. [PMID: 33267595 DOI: 10.17235/reed.2020.7431/2020] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
BACKGROUND AND AIM dermatological manifestations are normally found in one third of patients with inflammatory bowel disease. In this study, the prevalence, clinical characteristics, intestinal disease activity, and treatment response of neutrophilic dermatoses (pyoderma gangrenosum and Sweet´s syndrome) were determined in patients with inflammatory bowel disease. METHODS a retrospective, observational study was performed in patients with inflammatory bowel disease and neutrophilic dermatoses between March 2012 and March 2018. RESULTS of 444 patients analyzed, 10 complied with the inclusion criteria. Seven had pyoderma gangrenosum and three presented Sweet's syndrome; and one patient developed both pathologies. The prevalence of neutrophilic dermatoses was 2.3 % (10/444), comprising 1.6 % with pyoderma gangrenosum and 0.7 % with Sweet's syndrome. Six out of seven patients with pyoderma gangrenosum were female and had ulcerative colitis. The most frequent clinical presentation of pyoderma gangrenosum was the ulcerative subtype. Active moderate-to-severe intestinal disease was found in 71.4 % of patients. Biological therapy was prescribed to three patients with partial response to corticosteroids and persistent intestinal disease activity. This therapy was not indicated for cutaneous manifestations only. Three patients with moderate-to-severe Crohn´s disease presented classical (n = 2) and pustular (n = 1) Sweet's syndrome. A complete response was achieved in all Sweet's syndrome cases treated with corticosteroids. Biological therapy was prescribed to control intestinal disease activity. CONCLUSIONS pyoderma gangrenosum was the most frequent cutaneous manifestation of neutrophilic dermatoses, predominantly in females with ulcerative colitis, and highly associated with intestinal disease activity. Anti-tumor necrosis factor was effective in patients with partial cutaneous and intestinal disease response.
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Affiliation(s)
- Florencia Giraudo
- Gastroenterología, Hospital Interzonal General de Agudos (HIGA) "General San Martín", Argentina
| | - Eugenia Miraglia
- Dermatología, Hospital Interzonal General de Agudos (HIGA) "General San Martín", Argentina
| | - María Laura Garbi
- Gastroenterología, Hospital Interzonal General de Agudos (HIGA) "General San Martín", Argentina
| | - Martín Yantorno
- Gastroenterología, Hospital Interzonal General de Agudos (HIGA) "General San Martín", Argentina
| | - María Roxana Maradeo
- Dermatología, Hospital Interzonal General de Agudos (HIGA) "General San Martín", Argentina
| | - Gustavo Javier Correa
- Gastroenterologìa, Hospital Interzonal General de Agudos (HIGA) "General San Martín", Argentina
| | - Francisco Tufare
- Gastroenterologia, Hospital Interzonal General de Agudos (HIGA) "General San Martín", Argentina
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5
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Facheris P, De Santis M, Gargiulo L, Pavia G, Valenti M, Manara S, Borroni RG, Costanzo A, Narcisi A. Concomitant Pyoderma Gangrenosum-like and Amicrobial Pustulosis of the Folds: a Case Report. J Clin Immunol 2020; 40:1038-1040. [PMID: 32648026 PMCID: PMC7505873 DOI: 10.1007/s10875-020-00819-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2020] [Accepted: 06/30/2020] [Indexed: 11/29/2022]
Affiliation(s)
- Paola Facheris
- Department of Dermatology, Humanitas Clinical and Research Center-IRCCS, Via Manzoni 56, 20089, Rozzano, MI, Italy. .,Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, MI, Italy.
| | - Maria De Santis
- Department of Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center-IRCCS, Rozzano, MI, Italy
| | - Luigi Gargiulo
- Department of Dermatology, Humanitas Clinical and Research Center-IRCCS, Via Manzoni 56, 20089, Rozzano, MI, Italy.,Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, MI, Italy
| | - Giulia Pavia
- Department of Dermatology, Humanitas Clinical and Research Center-IRCCS, Via Manzoni 56, 20089, Rozzano, MI, Italy.,Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, MI, Italy
| | - Mario Valenti
- Department of Dermatology, Humanitas Clinical and Research Center-IRCCS, Via Manzoni 56, 20089, Rozzano, MI, Italy.,Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, MI, Italy
| | - Sofia Manara
- Department of Pathology, Humanitas Clinical and Research Center-IRCCS, Rozzano, MI, Italy
| | - Riccardo G Borroni
- Department of Dermatology, Humanitas Clinical and Research Center-IRCCS, Via Manzoni 56, 20089, Rozzano, MI, Italy.,Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, MI, Italy
| | - Antonio Costanzo
- Department of Dermatology, Humanitas Clinical and Research Center-IRCCS, Via Manzoni 56, 20089, Rozzano, MI, Italy.,Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, MI, Italy
| | - Alessandra Narcisi
- Department of Dermatology, Humanitas Clinical and Research Center-IRCCS, Via Manzoni 56, 20089, Rozzano, MI, Italy
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Wang EA, Steel A, Luxardi G, Mitra A, Patel F, Cheng MY, Wilken R, Kao J, de Ga K, Sultani H, Merleev AA, Marusina AI, Brassard A, Fung MA, Konia T, Shimoda M, Maverakis E. Classic Ulcerative Pyoderma Gangrenosum Is a T Cell-Mediated Disease Targeting Follicular Adnexal Structures: A Hypothesis Based on Molecular and Clinicopathologic Studies. Front Immunol 2018; 8:1980. [PMID: 29379508 PMCID: PMC5775228 DOI: 10.3389/fimmu.2017.01980] [Citation(s) in RCA: 44] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2017] [Accepted: 12/20/2017] [Indexed: 12/12/2022] Open
Abstract
Background Pyoderma gangrenosum (PG) is a debilitating ulcerative skin disease that is one of the most common associated diseases seen in patients with inflammatory bowel disease and rheumatoid arthritis. Although PG is classified as a neutrophilic dermatosis, its pathophysiology is poorly understood. Objective Use data obtained from patient-reported histories, immunohistochemistry, and gene expression analysis to formulate a hypothesis on PG pathophysiology. Methods Ten PG patients participated and answered questions about new ulcer formation. Skin biopsies of healed prior ulcers and adjacent normal skin were obtained from four patients for immunohistochemistry. Scars from healthy patients and patients with discoid lupus were used as additional controls. New onset PG papules were analyzed using immunohistochemistry and gene expression analysis via quantitative real-time PCR. Results All PG patients reported that healed sites of previous ulceration are refractory to re-ulceration. Simultaneous biopsies of healed and uninvolved skin triggered ulceration only in the latter. On immunohistochemistry, healed PG scars showed complete loss of pilosebaceous units, which were present in normal skin, and to a lesser extent in control scars, and discoid scars. Early PG papules showed perivascular and peripilosebaceous T cell infiltrates, rather than neutrophils. These early inflammatory events were dominated by increased gene expression of CXCL9, CXCL10, CXCL11, IL-8, IL-17, IFNG, and IL-36G and transcription factors consistent with Th1 phenotype. Limitations Small sample size was the main limitation. Conclusion We put forth the hypothesis that PG is a T cell response resulting in the destruction of pilosebaceous units.
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Affiliation(s)
- Elizabeth A Wang
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Andrea Steel
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Guillaume Luxardi
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Anupam Mitra
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Forum Patel
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Michelle Y Cheng
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Reason Wilken
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Jason Kao
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Kristopher de Ga
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Hawa Sultani
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Alexander A Merleev
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Alina I Marusina
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Alain Brassard
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Maxwell A Fung
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States.,Department of Pathology, University of California, Davis, Sacramento, CA, United States
| | - Thomas Konia
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States.,Department of Pathology, University of California, Davis, Sacramento, CA, United States
| | - Michiko Shimoda
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
| | - Emanual Maverakis
- Department of Dermatology, University of California, Davis, Sacramento, CA, United States
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Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease: Prevalence, Presentation, and Anti-TNF Treatment. J Pediatr Gastroenterol Nutr 2017; 65:200-206. [PMID: 27801751 DOI: 10.1097/mpg.0000000000001455] [Citation(s) in RCA: 79] [Impact Index Per Article: 9.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
BACKGROUND There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD). METHODS Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively. RESULTS A total of 55 patients (16.7%) experienced 1-4 EIM (39 Crohn disease, 12 ulcerative colitis, and 4 IBD-unclassified patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs 5.0%, P = 0.014). EIM were more frequent in Crohn disease when compared to ulcerative colitis/IBD-unclassified (22.5% vs 10.3%, P = 0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). Approximately 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (-37.5-149.0). Thirty-one of the 55 patients (56.4%) were treated with 1 or more anti-tumor necrosis factor (TNF) agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs 35.0%, P = 0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%). CONCLUSIONS In a cohort of pediatric patients with IBD, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings may translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti-TNF for the treatment of certain EIM is effective, although a substantial proportion of new EIM may present despite ongoing anti-TNF therapy.
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Anti-TNF Treatment for Extraintestinal Manifestations of Inflammatory Bowel Disease in the Swiss IBD Cohort Study. Inflamm Bowel Dis 2017; 23:1174-1181. [PMID: 28452862 DOI: 10.1097/mib.0000000000001109] [Citation(s) in RCA: 68] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Extraintestinal manifestations (EIMs) in patients with inflammatory bowel disease (IBD) are frequently observed. Little is known about the efficacy of anti-tumor necrosis factor (TNF) in EIM management. We assessed the effect of 3 anti-TNF agents (infliximab, adalimumab, and certolizumab pegol) on EIM evolution. METHODS Data on 1249 patients from the Swiss IBD Cohort Study (SIBDCS) were analyzed. All EIMs were diagnosed by relevant specialists. Response was classified into improvement, stable disease, and clinical worsening based on the physician's interpretation. RESULTS Of the 366 patients with at least 1 EIM, 213 (58.2%) were ever treated with an anti-TNF. A total of 299 treatments were started for 355 EIMs. Patients with EIM were significantly more often treated with anti-TNF compared with those without EIM (58.2% versus 21.0%, P < 0.001). Infliximab was the most frequently used drug (63.2%). In more than 71.8%, a clinical response of the underlying EIM to anti-TNF therapy was observed. In 92 patients (43.2%), anti-TNF treatments were started for the purpose of treating EIM rather than IBD. Response rates to anti-TNF were generally good and best for psoriasis, aphthous stomatitis, uveitis, and peripheral arthritis. In 11 patients, 14 EIM occurred under anti-TNF treatment. CONCLUSIONS Anti-TNF was frequently used among patients with EIM. In more than 40%, anti-TNF treatments are started to treat EIM rather than IBD. Given the good response rates, anti-TNF seems to be a valuable option in the treatment of EIM, whereas appearance of EIM under anti-TNF does not seem to be a source of considerable concern.
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9
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Greuter T, Navarini A, Vavricka SR. Skin Manifestations of Inflammatory Bowel Disease. Clin Rev Allergy Immunol 2017. [PMID: 28643285 DOI: 10.1007/s12016-017-8617-4] [Citation(s) in RCA: 67] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
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11
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Gupta AS, Ortega-Loayza AG. Ocular pyoderma gangrenosum: A systematic review. J Am Acad Dermatol 2016; 76:512-518. [PMID: 27836332 DOI: 10.1016/j.jaad.2016.08.049] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2016] [Revised: 08/22/2016] [Accepted: 08/23/2016] [Indexed: 01/06/2023]
Abstract
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.
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Affiliation(s)
- Angela S Gupta
- School of Medicine, Virginia Commonwealth University, Richmond, Virginia
| | - Alex G Ortega-Loayza
- Department of Dermatology, Oregon Health and Sciences University, Portland, Oregon.
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12
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Papadopoulos AJ, Schwartz RA, Kapila R, Samady JA, Ruszczak Z, Rao BK, Lambert WC. Pyoderma Vegetans. J Cutan Med Surg 2016. [DOI: 10.1177/120347540100500306] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
Background: Pyoderma vegetans is a rare condition that is clinically characterized by large verrucous plaques with elevated borders and multiple pustules. The etiology of this disorder remains unknown. Objectives: We describe a 24-year-old woman with rapidly evolving pyoderma vegetans. Our patient had the unique additional findings of a highly elevated serum IgE level and a history of hidradenitis suppurativa. Conclusions: Pyoderma vegetans is diagnosed on clinical and histological criteria. Differentiation must be made from disorders such as pyoderma gangrenosum, Sweet's syndrome, and deep fungal infections. We illustrate a case of pyoderma vegetans and review the literature on this rare disorder. Clinical and histological criteria for diagnosis are presented, as well as differentiation from some mimicking disorders.
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Affiliation(s)
| | - Robert A. Schwartz
- Dermatology, New Jersey Medical School, Newark, New Jersey
- Pathology, New Jersey Medical School, Newark, New Jersey
| | - Rajendra Kapila
- Infectious Diseases, New Jersey Medical School, Newark, New Jersey
| | | | | | | | - W. Clark Lambert
- Dermatology, New Jersey Medical School, Newark, New Jersey
- Pathology, New Jersey Medical School, Newark, New Jersey
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13
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Chronological Order of Appearance of Extraintestinal Manifestations Relative to the Time of IBD Diagnosis in the Swiss Inflammatory Bowel Disease Cohort. Inflamm Bowel Dis 2015; 21:1794-800. [PMID: 26020601 DOI: 10.1097/mib.0000000000000429] [Citation(s) in RCA: 162] [Impact Index Per Article: 16.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
BACKGROUND Data evaluating the chronological order of appearance of extraintestinal manifestations (EIMs) relative to the time of inflammatory bowel disease (IBD) diagnosis is currently lacking. We aimed to assess the type, frequency, and chronological order of appearance of EIMs in patients with IBD. METHODS Data from the Swiss Inflammatory Bowel Disease Cohort Study were analyzed. RESULTS The data on 1249 patients were analyzed (49.8% female, median age: 40 [interquartile range, 30-51 yr], 735 [58.8%] with Crohn's disease, 483 [38.7%] with ulcerative colitis, and 31 [2.5%] with indeterminate colitis). A total of 366 patients presented with EIMs (29.3%). Of those, 63.4% presented with 1, 26.5% with 2, 4.9% with 3, 2.5% with 4, and 2.7% with 5 EIMs during their lifetime. Patients presented with the following diseases as first EIMs: peripheral arthritis 70.0%, aphthous stomatitis 21.6%, axial arthropathy/ankylosing spondylitis 16.4%, uveitis 13.7%, erythema nodosum 12.6%, primary sclerosing cholangitis 6.6%, pyoderma gangrenosum 4.9%, and psoriasis 2.7%. In 25.8% of cases, patients presented with their first EIM before IBD was diagnosed (median time 5 mo before IBD diagnosis: range, 0-25 mo), and in 74.2% of cases, the first EIM manifested itself after IBD diagnosis (median: 92 mo; range, 29-183 mo). CONCLUSIONS In one quarter of patients with IBD, EIMs appeared before the time of IBD diagnosis. Occurrence of EIMs should prompt physicians to look for potential underlying IBD.
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14
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Hurabielle C, Schneider P, Baudry C, Bagot M, Allez M, Viguier M. Certolizumab pegol - A new therapeutic option for refractory disseminated pyoderma gangrenosum associated with Crohn's disease. J DERMATOL TREAT 2015; 27:67-9. [PMID: 25909366 DOI: 10.3109/09546634.2015.1034075] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
Abstract
Systemic steroids, in association or not with cyclosporin, are indicated for the treatment of large or widespread Pyoderma gangrenosum (PG). We report the case of a 27-year-old woman with a 15-year history of severe Crohn's disease, who developed a severe and disseminated PG, refractory to multiple lines of treatment. Infliximab and adalimumab were contraindicated, either because of allergy or of ineffectiveness on Crohn's disease. The addition of certolizumab pegol to the baseline treatment, associating systemic steroids and tacrolimus, finally allowed the complete healing of PG. Oral prednisone was stopped and tacrolimus was decreased, without any cutaneous or digestive relapse. Certolizumab pegol could be an alternative therapy in the treatment of PG in case of intolerance or ineffectiveness of the other anti-tumor necrosis factor (anti-TNF) therapies.
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Affiliation(s)
- Charlotte Hurabielle
- a Department of Dermatology , Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (APHP) , Paris , France
| | - Pierre Schneider
- a Department of Dermatology , Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (APHP) , Paris , France .,b Université Paris Diderot , Sorbonne Paris Cité , Paris , France , and
| | - Clotilde Baudry
- b Université Paris Diderot , Sorbonne Paris Cité , Paris , France , and.,c Department of Gastroenterology , Hôpital Saint-Louis , APHP , Paris , France
| | - Martine Bagot
- a Department of Dermatology , Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (APHP) , Paris , France .,b Université Paris Diderot , Sorbonne Paris Cité , Paris , France , and
| | - Matthieu Allez
- b Université Paris Diderot , Sorbonne Paris Cité , Paris , France , and.,c Department of Gastroenterology , Hôpital Saint-Louis , APHP , Paris , France
| | - Manuelle Viguier
- a Department of Dermatology , Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (APHP) , Paris , France .,b Université Paris Diderot , Sorbonne Paris Cité , Paris , France , and
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15
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DeFilippis E, Feldman S, Huang W. The genetics of pyoderma gangrenosum and implications for treatment: a systematic review. Br J Dermatol 2015; 172:1487-1497. [PMID: 25350484 DOI: 10.1111/bjd.13493] [Citation(s) in RCA: 63] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/10/2014] [Indexed: 12/22/2022]
Affiliation(s)
- E.M. DeFilippis
- Center for Dermatology Research; Department of Dermatology; Wake Forest School of Medicine, Medical Center Boulevard; Winston-Salem NC 27157 U.S.A
| | - S.R. Feldman
- Center for Dermatology Research; Department of Dermatology; Wake Forest School of Medicine, Medical Center Boulevard; Winston-Salem NC 27157 U.S.A
- Department of Pathology; Wake Forest School of Medicine, Medical Center Boulevard; Winston-Salem NC 27157 U.S.A
- Department of Public Health Sciences; Wake Forest School of Medicine, Medical Center Boulevard; Winston-Salem NC 27157 U.S.A
| | - W.W. Huang
- Center for Dermatology Research; Department of Dermatology; Wake Forest School of Medicine, Medical Center Boulevard; Winston-Salem NC 27157 U.S.A
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16
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Butts GT, Bishop PR, Wyatt JP, Nowicki MJ. Leukocytoclastic vasculitis in an adolescent with ulcerative colitis: Report of a case and review of the literature. SAGE Open Med Case Rep 2014; 2:2050313X14547609. [PMID: 27489650 PMCID: PMC4857370 DOI: 10.1177/2050313x14547609] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2014] [Accepted: 07/07/2014] [Indexed: 01/01/2023] Open
Abstract
An adolescent female with long-standing, difficult-to-control ulcerative colitis developed leukocytoclastic vasculitis, a rare cutaneous extra-intestinal manifestation of the inflammatory bowel disease. The authors provide a literature review on leukocytoclastic vasculitis complicating ulcerative colitis. Furthermore, the clinical features of leukocytoclastic vasculitis are compared and contrasted with the more common cutaneous extra-intestinal manifestations of inflammatory bowel disease, erythema nodosum, and pyoderma gangrenosum.
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Affiliation(s)
- G Tyler Butts
- School of Medicine, University of Mississippi Medical Center, Jackson, MS, USA
| | - Phyllis R Bishop
- Division of Pediatric Gastroenterology, University of Mississippi Medical Center, Jackson, MS, USA
| | - Julie P Wyatt
- Department of Dermatology, University of Mississippi Medical Center, Jackson, MS, USA
| | - Michael J Nowicki
- Division of Pediatric Gastroenterology, University of Mississippi Medical Center, Jackson, MS, USA
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17
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Autoinflammatory skin disorders in inflammatory bowel diseases, pyoderma gangrenosum and Sweet's syndrome: a comprehensive review and disease classification criteria. Clin Rev Allergy Immunol 2014; 45:202-10. [PMID: 23334898 DOI: 10.1007/s12016-012-8351-x] [Citation(s) in RCA: 90] [Impact Index Per Article: 8.2] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Pyoderma gangrenosum (PG) and Sweet's syndrome (SS) are skin diseases usually presenting with recurrent ulcers and erythematous plaques, respectively. The accumulation of neutrophils in the skin, characteristic of these conditions, led to coin the term of neutrophilic dermatoses to define them. Recently, neutrophilic dermatoses have been included in the group of autoinflammatory diseases, which classically comprises genetically determined forms due to mutations of genes regulating the innate immune response. Both PG and SS are frequently associated with inflammatory bowel diseases (IBDs); however, IBD patients develop PG in 1-3 % of cases, whereas SS is rarer. Clinically, PG presents with deep erythematous-to-violaceous painful ulcers with well-defined borders; bullous, pustular, and vegetative variants can also occur. SS is characterized by the abrupt onset of fever, peripheral neutrophilia, tender erythematous skin lesions, and a diffuse neutrophilic dermal infiltrate. It is also known as acute febrile neutrophilic dermatosis. Treatment of PG involves a combination of wound care, topical medications, antibiotics for secondary infections, and treatment of the underlying IBD. Topical therapies include corticosteroids and the calcineurin inhibitor tacrolimus. The most frequently used systemic medications are corticosteroids and cyclosporine, in monotherapy or in combination. Dapsone, azathioprine, cyclophosphamide, methotrexate, intravenous immunoglobulins, mycophenolate mofetil, and plasmapheresis are considered second-line agents. Hyperbaric oxygen, as supportive therapy, can be added. Anti-TNF-α agents such as etanercept, infliximab, and adalimumab are used in refractory cases. SS is usually responsive to oral corticosteroids, and the above-mentioned immunosuppressants should be considered in resistant or highly relapsing cases.
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18
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Cutaneous manifestations in patients with inflammatory bowel diseases: pathophysiology, clinical features, and therapy. Inflamm Bowel Dis 2014; 20:213-27. [PMID: 24105394 DOI: 10.1097/01.mib.0000436959.62286.f9] [Citation(s) in RCA: 78] [Impact Index Per Article: 7.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
The skin is one of the most common extraintestinal organ system affected in patients with inflammatory bowel disease (IBD), including both Crohn's disease and ulcerative colitis. The skin manifestations associated with IBD are polymorphic and can be classified into 4 categories according to their pathophysiology: (1) specific, (2) reactive, (3) associated, and (4) induced by IBD treatment. Cutaneous manifestations are regarded as specific if they share with IBD the same granulomatous histopathological pattern: perianal or metastatic Crohn's disease (commonly presenting with abscesses, fistulas or hidradenitis suppurativa-like features) is the prototype of this setting. Reactive cutaneous manifestations are different from IBD in the histopathology but have close physiopathological links: pyoderma gangrenosum, a neutrophil-mediated autoinflammatory skin disease typically manifesting as painful ulcers, is the paradigm of this group. Among the cutaneous diseases associated with IBD, the most commonly seen are erythema nodosum, a form of panniculitis most commonly involving bilateral pretibial areas, and psoriasis, a T helper 1/T helper 17-mediated erythematous squamous inflammatory disease. Finally, the number of cutaneous adverse reactions because of IBD therapies is progressively increasing. The most frequent drug-induced cutaneous manifestations are psoriasis-like, eczema-like, and lichenoid eruptions, as well as cutaneous lupus erythematosus for biologics, and nonmelanoma skin cancer, mainly basal cell and squamous cell carcinomas for thiopurines.
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19
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Zippi M, Pica R, De Nitto D, Paoluzi P. Biological therapy for dermatological manifestations of inflammatory bowel disease. World J Clin Cases 2013; 1:74-78. [PMID: 24303470 PMCID: PMC3845939 DOI: 10.12998/wjcc.v1.i2.74] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2013] [Revised: 02/18/2013] [Accepted: 03/29/2013] [Indexed: 02/05/2023] Open
Abstract
Ulcerative colitis and Crohn’s disease are the two forms of inflammatory bowel disease (IBD). The advent of biological drugs has significantly changed the management of these conditions. Skin manifestations are not uncommon in IBD. Among the reactive lesions (immune-mediated extraintestinal manifestations), erythema nodosum (EN) and pyoderma gangrenosum (PG) are the two major cutaneous ills associated with IBD, while psoriasis is the dermatological comorbidity disease observed more often. In particular, in the last few years, anti-tumor necrosis factor (TNF)-α agents have been successfully used to treat psoriasis, especially these kinds of lesions that may occur during the treatment with biological therapies. The entity of the paradoxical manifestations has been relatively under reported as most lesions are limited and a causal relationship with the treatment is often poorly understood. The reason for this apparent side-effect of the therapy still remains unclear. Although side effects may occur, their clinical benefits are undoubted. This article reviews the therapeutic effects of the two most widely used anti-TNF-α molecules, infliximab (a fusion protein dimer of the human TNF-α receptor) and adalimumab (a fully human monoclonal antibody to TNF-α), for the treatment of the major cutaneous manifestations associated with IBD (EN, PG and psoriasis).
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20
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Huang BL, Chandra S, Shih DQ. Skin manifestations of inflammatory bowel disease. Front Physiol 2012; 3:13. [PMID: 22347192 PMCID: PMC3273725 DOI: 10.3389/fphys.2012.00013] [Citation(s) in RCA: 124] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2011] [Accepted: 01/17/2012] [Indexed: 12/13/2022] Open
Abstract
Inflammatory bowel disease (IBD) is a disease that affects the intestinal tract via an inflammatory process. Patients who suffer from IBD often have diseases that affect multiple other organ systems as well. These are called extraintestinal manifestations and can be just as, if not more debilitating than the intestinal inflammation itself. The skin is one of the most commonly affected organ systems in patients who suffer from IBD. The scientific literature suggests that a disturbance of the equilibrium between host defense and tolerance, and the subsequent over-activity of certain immune pathways are responsible for the cutaneous disorders seen so frequently in IBD patients. The purpose of this review article is to give an overview of the types of skin diseases that are typically seen with IBD and their respective pathogenesis, proposed mechanisms, and treatments. These cutaneous disorders can manifest as metastatic lesions, reactive processes to the intestinal inflammation, complications of IBD itself, or side effects from IBD treatments; these can be associated with IBD via genetic linkage, common autoimmune processes, or other mechanisms that will be discussed in this article. Ultimately, it is important for healthcare providers to understand that skin manifestations should always be checked and evaluated for in patients with IBD. Furthermore, skin disorders can predate gastrointestinal symptoms and thus may serve as important clinical indicators leading physicians to earlier diagnosis of IBD.
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Affiliation(s)
- Brian L Huang
- Department of Medicine, Cedars-Sinai Medical Center Los Angeles, CA, USA
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21
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Vavricka SR, Brun L, Ballabeni P, Pittet V, Prinz Vavricka BM, Zeitz J, Rogler G, Schoepfer AM. Frequency and risk factors for extraintestinal manifestations in the Swiss inflammatory bowel disease cohort. Am J Gastroenterol 2011; 106:110-9. [PMID: 20808297 DOI: 10.1038/ajg.2010.343] [Citation(s) in RCA: 360] [Impact Index Per Article: 25.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVES Data on the frequency of extraintestinal manifestations (EIMs) in Crohn's disease (CD) and ulcerative colitis (UC) and analyses of their risk factors are scarce. We evaluated their prevalence and risk factors in a large nationwide cohort of inflammatory bowel disease (IBD) patients. METHODS IBD patients from an adult clinical cohort in Switzerland (Swiss IBD cohort study) were prospectively included. Data from validated physician enrolment questionnaires were analyzed. RESULTS A total of 950 patients were included, 580 (61%) with CD (mean age 41 years) and 370 (39%) with UC (mean age 42 years). Of these, 249 (43%) of CD and 113 (31%) of UC patients had one to five EIMs. The following EIMs were found: arthritis (CD 33%, UC 21%), aphthous stomatitis (CD 10%, UC 4%), uveitis (CD 6%, UC 4%), erythema nodosum (CD 6%, UC 3%), ankylosing spondylitis (CD 6%, UC 2%), psoriasis (CD 2%, UC 1%), pyoderma gangrenosum (CD and UC each 2%), and primary sclerosing cholangitis (CD 1%, UC 4%). Multiple logistic regression identified the following risk factors for ongoing EIM in CD: active disease (odds ratio (OR)=1.95, 95% confidence interval (CI)=1.17-3.23, P=0.01), and positive IBD family history (OR=1.77, 95% CI=1.07-2.92, P=0.025). No risk factors were identified in UC patients. CONCLUSIONS EIMs are a frequent problem in CD and UC patients. Active disease and positive IBD family history are associated with ongoing EIM in CD patients. Identification of EIM prevalence and associated risk factors may result in increased awareness for this problem and thereby facilitating their diagnosis and therapeutic management.
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Affiliation(s)
- Stephan R Vavricka
- Division of Gastroenterology, Department of Internal Medicine, University Hospital, Zurich, Switzerland.
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22
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Kaplan R, Baitoo H, Kuchera E. Peristomal pyoderma gangrenosum. J DERMATOL TREAT 2009. [DOI: 10.3109/09546639309084520] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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23
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Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venereol 2009; 23:1008-17. [PMID: 19470075 DOI: 10.1111/j.1468-3083.2009.03199.x] [Citation(s) in RCA: 299] [Impact Index Per Article: 18.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Pyoderma gangrenosum is a rare, ulcerative, cutaneous condition. First described in 1930, the pathogenesis of pyoderma gangrenosum remains unknown, but it is probably related to a hyperergic reaction. There are various clinical and histological variants of this disorder. Pyoderma gangrenosum often occurs in association with a systemic disease such as inflammatory bowel disease, rheumatologic disease, paraproteinaemia, or haematological malignancy. The diagnosis, mainly based on the clinical presentation and course, is confirmed through a process of elimination of other causes of cutaneous ulcers. Local treatment may be sufficient for mild disease, while for severe cases, systemic immunosuppressants are the mainstay. Long-term treatment with these agents is often required, but this can expose patients to adverse side-effects.
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Affiliation(s)
- E Ruocco
- Department of Dermatology, Second University of Naples, Naples, Italy
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24
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Abstract
Ulcerative colitis (UC) is an inflammatory disorder of the colon that is associated with several extraintestinal manifestations in multiple organs. Several mucous membrane and skin disorders occur in patients with UC. These disorders are not unique to UC and often occur secondary to other causes or in the absence of an apparent cause. One or more such disorders may occur together in association with UC. Mucous membrane and skin disorders may antedate, occur with, or postdate the onset of UC. The dermatologist plays an important role in suspecting the diagnosis of UC that presents with associated mucous membrane or skin disorders. This review covers the clinical presentation, differential diagnosis, workup, and management of selected mucocutaneous manifestations in UC.
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Affiliation(s)
- Shereen Timani
- Department of Dermatology, College of Medicine, University of Cincinnati, Cincinnati, OH 45267-0592, USA
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25
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Aseni P, Sandro SD, Mihaylov P, Lamperti L, Carlis LGD. Atypical presentation of pioderma gangrenosum complicating ulcerative colitis: Rapid disappearance with methylprednisolone. World J Gastroenterol 2008; 14:5471-3. [PMID: 18803362 PMCID: PMC2744162 DOI: 10.3748/wjg.14.5471] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Piodermal gangrenosum (PG) is an uncommon ulcerative cutaneous dermatosis associated with a variety of systemic diseases, including inflammatory bowel disease (IBD), arthritis, leukaemia, hepatitis, and primary biliary cirrhosis. Other cutaneous ulceration resembling PG had been described in literature. There has been neither laboratory finding nor histological feature diagnostic of PG, and diagnosis of PG is mainly made based on the exclusion criteria. We present here a patient, with ulcerative colitis (UC) who was referred to the emergency section with a large and rapidly evolving cutaneous ulceration. Laboratory and microbiological investigation associated with histological findings of the ulcer specimen allowed us to exclude autoimmune and systemic diseases as well as immuno-proliferative disorders. An atypical presentation of PG with UC was diagnosed. Pulse boluses of i.v. methyl-prednisolone were started, and after tapering steroids, complete resolution of the skin lesion was achieved in 3 wk. The unusual rapid healing of the skin ulceration with steroid mono-therapy and the atypical cutaneous presentation in this patient as well as the risk of misdiagnosis of PG in the clinical practice were discussed.
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26
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27
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Ruhl AP, Ganz JE, Bickston SJ. Neutrophilic folliculitis and the spectrum of pyoderma gangrenosum in inflammatory bowel disease. Dig Dis Sci 2007; 52:18-24. [PMID: 17160479 DOI: 10.1007/s10620-006-9361-4] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/02/2005] [Accepted: 03/28/2006] [Indexed: 01/06/2023]
Affiliation(s)
- A P Ruhl
- University of Virginia School of Medicine, Charlottesville, Virginia, USA
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28
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Nakamura T, Yagi H, Kurachi K, Suzuki S, Konno H. Intestinal Behcet’s disease with pyoderma gangrenosum: A case report. World J Gastroenterol 2006; 12:979-81. [PMID: 16521233 PMCID: PMC4066170 DOI: 10.3748/wjg.v12.i6.979] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
We report here a very rare case of intestinal Behcet’s disease with pyoderma gangrenosum. A 16-year-old woman who was diagnosed with intestinal Behcet’s disease by the presence of cutaneous pathergy together with two major criteria (oral and genital aphthoses) and one minor criterion (gastrointestinal manifestations), was referred to our hospital with a left lower leg ulcer and abdominal pain in September 1989. Colonoscopy demonstrated flare-up colitis involving the entire colon. Her lower leg lesion was a painful destructive ulcer with an irregular margin and a ragged overhanging edge. Based on these clinical and laboratory findings, we diagnosed her cutaneous ulcer as pyoderma gangrenosum developing with exacerbated intestinal Behcet’s disease. Her cutaneous and intestinal lesions were poorly controlled though she received oral prednisolone treatment for a month. Because of aggravated abdominal symptoms with peritoneal irritation, we performed total colectomy in November 1989. The resected specimen was histologically compatible with intestinal Behcet’s disease showing severe inflammation with deep ulcerations and neutrophil accumulation. Subsequently, pyoderma gangrenosum rapidly improved. This clinical course may suggest the close relationship between pyoderma gangrenosum and intestinal Behcet’s disease.
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Affiliation(s)
- Toshio Nakamura
- The Second Department of Surgery, Hamamatsu University School of Medicine, 1-20-1 Handa-yama, Hamamatsu 431-3192, Japan.
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29
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Abstract
Inflammatory bowel disease (IBD) has many extraintestinal manifestations. Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. Anyone presenting with IBD should be examined for cutaneous manifestations. Pyoderma gangrenosum is a severe painful ulcerating disease that requires moist wound management and, in the absence of secondary infection, systemic corticosteroids, cyclosporine, or both. Infliximab may also be used. Erythema nodosum is a common cause of tender red nodules of the shins. Management includes leg elevation, NSAIDs, and potassium iodide. Oral manifestations of IBD include aphthous stomatitis, mucosal nodularity (cobblestoning), and pyostomatitis vegetans. Treatment should be directed both at the cutaneous lesions and at the underlying systemic condition.
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Affiliation(s)
- L B Trost
- Department of Dermatology, A61, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44195, USA.
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30
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Campbell S, Cripps S, Jewell DP. Therapy Insight: pyoderma gangrenosum—old disease, new management. ACTA ACUST UNITED AC 2005; 2:587-94. [PMID: 16327838 DOI: 10.1038/ncpgasthep0339] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2005] [Accepted: 09/27/2005] [Indexed: 11/08/2022]
Abstract
Well-designed studies that help guide physicians to apply the optimal therapeutic strategy for the management of pyoderma gangrenosum are lacking in the literature. A multidisciplinary approach is paramount for the effective management of this condition, with close involvement of a wound-care specialist and a microbiologist. Treatment should be stepwise in nature. Local wound care, avoidance of trauma and the application of local steroid or tacrolimus ointment are the first-line treatments. Steroid therapy is the most widely published effective therapy for achieving resolution of pyoderma gangrenosum, although there is growing evidence for the efficacy of infliximab in refractory cases. Other therapies such as dapsone and clofazamine should be left as third-line agents for refractory pyoderma gangrenosum, while novel treatments such as granulocyte apheresis should only be used under trial conditions, to gain an objective evaluation of their efficacy. This article reviews the published treatment strategies in current use, and aims to guide the effective management of pyoderma gangrenosum.
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Affiliation(s)
- Simon Campbell
- Pharmacy Department, John Radcliffe Hospital, Headington, Oxford, UK.
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31
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Krauze E, Brzezińska-Wcisło L, Kamińska-Winciorek G, Wygledowska-Kania M, Sygula E. Pyoderma gangrenosum coexisting with acute myelogenous leukaemia. J Eur Acad Dermatol Venereol 2005; 19:589-92. [PMID: 16164714 DOI: 10.1111/j.1468-3083.2005.01202.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
The frequency of occurrence of malignant neoplasms in the cases of pyoderma gangrenosum is not exactly determined, but it can be assessed to be at 7%. The aim of the study was to report a 26-year-old male patient with pyoderma gangrenosum coexisting with acute myelogenous leukaemia. The first skin lesions on both tibia occurred in June 2001. Prior to the proper diagnosis of pyoderma gangrenosum, the patient was treated surgically. Because of the dramatic dermatological and general condition in November 2001, the patient was admitted to the Dermatological Department of the Silesian Medical Academy in Katowice where the diagnosis of pyoderma gangrenosum was established. On the clinical and biochemical picture, the diagnosis of pyoderma gangrenosum within acute myelogenous leukaemia was made. Initially, cyclosporin A 200 mg orally per day in the therapy of pyoderma gangrenosum was administered to achieve a slight clinical improvement. Although chemotherapy leukaemia was performed, the patient died after 4 months of the confirmation of the acute myelogenous leukaemia diagnosis.
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Affiliation(s)
- E Krauze
- Dermatological Departament of Silesian Medical Academy, 40-027 Katowice, ul. Francuska 20/24, Poland.
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32
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Abstract
Pyoderma gangrenosum is a rare debilitating skin disease of unknown aetiology that manifests itself as painful lesions and is often associated with inflammatory bowel disease. Stoma care nurses will encounter it directly around the stoma as peristomal pyoderma gangrenosum (PPG). There are many case reports of varying methods of treatment for PPG, although none have proven to be efficacious in all circumstances. When these lesions develop in the peristomal area, treatment must be balanced with the need to maintain an intact stoma appliance.
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33
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Abstract
Pyoderma gangrenosum (PG) is a non-infectious reactive neutrophilic dermatosis which typically starts with pustules which rapidly evolve to painful ulcers of variable size and depth with undermined violaceous borders. Since its first description in 1930, the pathogenesis of PG has remained elusive even as an ever-widening range of systemic diseases has been described in association with it. The diagnosis of PG is based on clinical and pathologic features and requires exclusion of other conditions that produce ulcerations, since misdiagnosis exposes patients to risks associated with treatment. Critical to proper management are correct diagnosis, identification and treatment of any underlying disorder, and the appropriate choice of topical and systemic therapy. PG has four distinctive clinical and histologic variants, and the specific clinical features of the lesion may provide a clue to the associated disease. The most common associated diseases are inflammatory bowel disease, rheumatological or hematological disease or malignancy. Although there is no single successful treatment for PG, certain type of PG lesions are recognized to respond more readily to accepted therapies than others. Local treatment may be sufficient for mild disease, while systemic immunosuppressive therapy is necessary for severe cases. The treatments with the best clinical evidence are oral or pulse intravenous corticosteroids, and cyclosporine. Surgical therapy is useful in selected cases in conjunction with immunosuppression. Wound stabilization is obtained only through control of the systemic and local inflammatory process. Emerging therapies include use of platelet-derived growth factor and cell culture grafts when re-epithelialization is slow, and the TNF-alpha blocking agent infliximab for refractory disease. Despite advances in therapy, the long-term outcome for patients with PG remains unpredictable, because relapses are common.
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Affiliation(s)
- Curdin Conrad
- Department of Dermatology, University Hospital of Zurich
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34
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Cevik R, Nas K, Gur A, Ayyildiz O, Arica M. Small Joint Monoarthritis and Pyoderma Gangrenosum in Newly Diagnosed Ulcerative Colitis: An Unusual Presentation. J Clin Rheumatol 2004; 10:360-2. [PMID: 17043551 DOI: 10.1097/01.rhu.0000147140.70015.f5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Affiliation(s)
- Remzi Cevik
- Department of Physical Medicine & Rehabilitation University of Dicle School of Medicine Diyarbakir, Turkey (Cevik, Nas, Gur) Department of Internal Medicine University of Dicle School of Medicine Diyarbakir, Turkey (Ayyildiz) Department of Dermatology University of Dicle School of Medicine Diyarbakir, Turkey (Arica)
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35
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Cabral VLR, Miszputen SJ, Catapani WR. Anticorpo anticitoplasma de neutrófilos (ANCA) em pioderma gangrenoso, um marcador sorológico para associação com doenças sistêmicas: estudo de oito casos. An Bras Dermatol 2004. [DOI: 10.1590/s0365-05962004000100004] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
FUNDAMENTOS: A etiopatogenia da retocolite ulcerativa inespecífica (RCUI) e de suas manifestações extra-intestinais permanece em discussão, embora o envolvimento do sistema imune seja enfatizado, e uma possível participação dos neutrófilos é demonstrada pela detecção do anticorpo anticitoplasma de neutrófilo (ANCA) nessa doença inflamatória intestinal. O pioderma gangrenoso (PG) é considerado manifestação cutânea rara da retocolite ulcerativa, e o Anca também tem sido detectado nessa dermatose. OBJETIVOS: Investigar a relação entre o comportamento clínico da RCUI e o aparecimento do PG e sua associação com ANCA. CASUÍSTICA E MÉTODOS: Anca foi pesquisado nos soros de oito pacientes com PG, quatro apresentando RCUI, e os outros, PG não associado a doenças sistêmicas. RESULTADOS: Não se detectou o Anca nos soros dos portadores exclusivamente de pioderma gangrenoso. Dois casos de pancolite em atividade inflamatória acompanhada de pioderma e colangite esclerosante primária (CEP) apresentaram positividade para ANCA, enquanto os soros de dois outros pacientes com RCUI e PG tiveram resultados negativos. CONCLUSÕES: A Presença de ANCA nos soros de pacientes com PG associado a RCUI e CEP sugere que a associação com CEP seja responsável pela positividade do ANCA na presente amostra.
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Abstract
Since its first description in 1930, the pathogenesis of pyoderma gangrenosum (PG) has remained obscure even as an ever-widening array of systemic diseases has been described in association with it. The histopathologic distinction of PG from other ulcerative processes with dermal neutrophilia is challenging and at times impossible. In consequence, when confronted with a biopsy from such a lesion, the pathologist has an obligation to obtain a full and detailed clinical history. In short, as a diagnosis of PG does not hinge exclusively upon the biopsy findings in isolation from other studies, a solid knowledge of the clinical features, the systemic disease associations and the differential diagnosis will help the pathologist to avoid diagnostic pitfalls or the generation of a report which is non-contributory to patient care. In this review, we describe in detail the different clinicopathologic forms of PG, summarize the diseases associated with this process in the literature and in our experience, and briefly review the treatment options.
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Affiliation(s)
- A Neil Crowson
- Department of Dermatology, University of Oklahoma, Tulsa, OK, USA.
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Christodoulou DK, Katsanos KH, Kitsanou M, Stergiopoulou C, Hatzis J, Tsianos EV. Frequency of extraintestinal manifestations in patients with inflammatory bowel disease in Northwest Greece and review of the literature. Dig Liver Dis 2002; 34:781-6. [PMID: 12546513 DOI: 10.1016/s1590-8658(02)80071-8] [Citation(s) in RCA: 51] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND Inflammatory bowel disease mainly affects the bowel but also has extraintestinal manifestations. AIMS To report the frequency of extraintestinal manifestations in patients with inflammatory bowel disease in Northwest Greece. PATIENTS; The data files of 256 inflammatory bowel disease patients (diagnosis between 1983-19971 were analysed. METHODS Retrospective investigation of patient files. RESULTS In patients with ulcerative colitis: 13.9% (30/215) had developed skin manifestations, 6% (13/215) had kidney stones, 1.39% (3/215) had iridocyclitis, 1.86% (4/215) had primary sclerosing cholangitis, 4.18% (9/215) had sacroiliitis, 8.31% (18/215) had peripheral arthalgias, 2.3% (5/215) had colitic arthritis and finally 1.39% (3/215) had deep vein thrombosis). In patients with Crohn's disease: 24.3% (9/37) had developed skin manifestations, 5.4% (2/37) had kidney stones, 2.7% (1/37) had iridocyclitis, 16.2% (6/37) had sacroiliitis, 8.1% (3/37) had peripheral arthralgias, 5.4% (2/37) had colitic arthritis and, finally, 8.1% (3/37) had deep vein thrombosis. Sacroiliitis (p = 0.01), deep vein thrombosis (p = 0.04) and erythmema nodosum (p = 0.01) were more common in patients with Crohn's disease. CONCLUSIONS Extraintestinal manifestations are not rare in patients with inflammatory bowel disease, especially in Crohn's disease patients, in our area, but have, generally, a mild profile.
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Affiliation(s)
- D K Christodoulou
- Division of Internal Medicine (Hepato-Gastroenterology Unit), Medical School, University of Ioannina, Greece
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38
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Abstract
Lower extremity ulcers can be challenging diagnostically and therapeutically. This article, provides an overview of the different kinds of lower extremity wounds typically seen by the medical dermatologist. It also reviews new treatment modalities, including topical growth factors and bioengineered skin. A team approach is emphasized.
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Affiliation(s)
- M M Choucair
- Department of Dermatology, Henry Ford Health System, Detroit, Michigan, USA
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39
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Hagman JH, Carrozzo AM, Campione E, Romanelli P, Chimenti S. The use of high-dose immunoglobulin in the treatment of pyoderma gangrenosum. J DERMATOL TREAT 2001; 12:19-22. [PMID: 12171682 DOI: 10.1080/095466301750163527] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/17/2022]
Abstract
BACKGROUND Immunosuppressive medications such as corticosteroids and cyclosporin are the most commonly employed therapies in pyoderma gangrenosum. We describe a patient with multiple ulcers of pyoderma gangrenosum on the lower extremities in whom immunosuppressive therapy caused serious side effects and had to be discontinued but who was subsequently treated successfully with high dose intravenous immunoglobulin (IVIG). METHODS IVIG was given intravenously at a dose of 400 mg/kg per day for 5 consecutive days. After 1 week there was an arrest in the progression of the ulcers and a marked reduction in pain. Two weeks later clinical improvement of the ulcers was observed. Subsequently, IVIG was given at a dose of 1 g/kg per day for 2 consecutive days. RESULTS The treatment induced a dramatic clinical improvement of one ulcer and healing of the others. Side effects were minimal and well tolerated, and consisted of chills and a slight fever, which resolved with the administration of acetaminophen. CONCLUSION We feel that IVIG can be used in patients with pyoderma gangrenosum in whom conventional therapies are ineffective or produce serious side effects.
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Affiliation(s)
- J H Hagman
- University of Rome 'Tor Vergata', Department of Dermatology, Rome, Italy
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40
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Abstract
It has been claimed that pyoderma gangrenosum (PG) lesions may contain granulomatous foci when associated with Crohn's disease. To test this assertion, we obtained clinical histories and archived cutaneous biopsies from 34 PG patients. Thirteen of these patients had inflammatory bowel disease (IBD). Immunostaining with PGM1, a macrophage marker, revealed well-formed giant cells with three or more nuclei in biopsies from 6 of 13 patients with IBD. Five of the 6 biopsies came from patients with Crohn's disease and one from a patient with ulcerative colitis. Two were peristomal. In the 21 patients who had PG without IBD, no giant cells were seen. Thus, PGM1+ histiocytic giant cells within a PG lesion may be indicative of associated IBD (p = 0.006), particularly Crohn's disease.
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Affiliation(s)
- S Sanders
- Department of Dermatology, Cornell University Medical Center, New York, New York 10021, USA
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41
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Alam M, Grossman ME, Schneiderman PI, Blume RS, Benvenisty AI. Surgical management of pyoderma gangrenosum: case report and review. Dermatol Surg 2000; 26:1063-6. [PMID: 11096397 DOI: 10.1046/j.1524-4725.2000.0260111063.x] [Citation(s) in RCA: 21] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
BACKGROUND Commonly used treatments for pyoderma gangrenosum are medical, with immunosuppressive agents employed most often. OBJECTIVE To report a case and discuss the indications for radical surgical treatment of pyoderma gangrenosum. METHODS Analysis of a case of Crohn's disease-associated pyoderma gangrenosum treated with immunosuppression followed by amputation, and a review of the literature on surgical management of pyoderma gangrenosum. RESULTS In unstable patients with intractable multiple medical problems, surgical treatment of pyoderma gangrenosum may be indicated by the existence of these life-threatening comorbidities. The recent literature suggests that surgical management of pyoderma gangrenosum may also be appropriate in other special circumstances. CONCLUSIONS Surgical management, including amputation, may have a role in the management of pyoderma gangrenosum. Further research is needed to delineate precisely the circumstances and patient factors that are appropriate indications for such surgery.
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Affiliation(s)
- M Alam
- Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, New York, USA.
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42
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Lyon CC, Smith AJ, Beck MH, Wong GA, Griffiths CE. Parastomal pyoderma gangrenosum: Clinical features and management. J Am Acad Dermatol 2000. [DOI: 10.1067/mjd.2000.104515] [Citation(s) in RCA: 75] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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43
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Murata I, Satoh K, Yoshikawa I, Masumoto A, Sasaki E, Otsuki M. Recurrent subcutaneous abscess of the sternal region in ulcerative colitis. Am J Gastroenterol 1999; 94:844-5. [PMID: 10086677 DOI: 10.1111/j.1572-0241.1999.00957.x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
An 18-yr-old female patient with extensive ulcerative colitis suffered from several episodes of recurrent aseptic subcutaneous abscesses of the sternal region with a course paralleling that of her colitis. The abscess seemed to occur secondarily to osteomyelitis of the sternum, which is a manifestation of the synovitis, acne, pustulosis, hyperostosis, and osteomyelitis (SAPHO) syndrome.
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Affiliation(s)
- I Murata
- Third Department of Internal Medicine, University of Occupational and Environmental Health, and The Kyoritsu Hospital, Kitakyushu, Japan
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44
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Borum ML, Cannava M, Myrie-Williams C. Refractory, disfiguring vulvar pyoderma gangrenosum and Crohn's disease. Dig Dis Sci 1998; 43:720-2. [PMID: 9558025 DOI: 10.1023/a:1018849627342] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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46
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Affiliation(s)
- P Lionetti
- Dipartimento di Pediatria, Università di Firenze, Ospedale Meyer, Italy
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48
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Abstract
Pyoderma gangrenosum (PG) has four distinctive clinical and histologic variants. Some have morphologic and histologic overlapping features with other reactive neutrophilic skin conditions. PG often occurs in association with a systemic disease, and the specific clinical features of the skin lesion may provide a clue to the associated disease. Management of PG depends on its type and severity and usually requires aggressive local and systemic treatment.
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Affiliation(s)
- F C Powell
- Regional Centre of Dermatology, Mater Misericordiae, Dublin, Ireland
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49
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Nelson EA, Gibson B. The management of leg ulcers in a patient with ulcerative colitis. J Wound Care 1995; 4:391-393. [DOI: 10.12968/jowc.1995.4.9.391] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- EA Nelson
- A lecturer, Department of Nursing, University of Liverpool
| | - B. Gibson
- Leg ulcer specialist nurse, Falkirk and District Royal Infirmary
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50
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Abstract
PURPOSE Parastomal pyoderma gangrenosum is uncommon and its association with inflammatory bowel disease is unclear. This is a review of five patients with parastomal pyoderma gangrenosum. METHODS A retrospective review of five patients with ulcerative colitis (two patients) or Crohn's disease (three patients) who have been seen in one surgical unit was conducted. RESULTS All patients were females and each presented within nine months of abdominal surgery and stoma construction. All had active proctitis (n = 3) or perianal Crohn's disease (n = 2). Both patients with perianal Crohn's disease had a mild clinical course with healing of parastomal pyoderma gangrenosum when treated with steroids with and without low-dose cyclosporin A. They both had curettage of the perineal wound as well. In the remaining three patients with active proctitis, the parastomal lesions failed to resolve despite high-dose systemic steroids. By contrast, the parastomal pyoderma gangrenosum healed promptly in two of these patients following proctectomy for active proctitis. CONCLUSION The variable clinical outcome of parastomal pyoderma gangrenosum may be related to the activity of the underlying inflammatory bowel disease or possibly to low-grade perineal sepsis.
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Affiliation(s)
- J J Tjandra
- Department of Surgery, University of Wales College of Medicine, Cardiff, United Kingdom
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