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      Associated factors and comorbidities in patients with pyoderma gangrenosum in Germany: a retrospective multicentric analysis in 259 patients

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          Abstract

          Background

          Pyoderma gangrenosum (PG) is a rarely diagnosed ulcerative neutrophilic dermatosis with unknown origin that has been poorly characterized in clinical studies so far. Consequently there have been significant discussions about its associated factors and comorbidities. The aim of our multicenter study was to analyze current data from patients in dermatologic wound care centers in Germany in order to describe associated factors and comorbidities in patients with PG.

          Methods

          Retrospective clinical investigation of patients with PG from dermatologic wound care centers in Germany.

          Results

          We received data from 259 patients with PG from 20 different dermatologic wound care centers in Germany. Of these 142 (54.8%) patients were female, 117 (45.2%) were male; with an age range of 21 to 95 years, and a mean of 58 years. In our patient population we found 45.6% with anemia, 44.8% with endocrine diseases, 12.4% with internal malignancies, 9.3% with chronic inflammatory bowel diseases and 4.3% with elevated creatinine levels. Moreover 25.5% of all patients had a diabetes mellitus with some aspects of potential association with the metabolic syndrome.

          Conclusions

          Our study describes one of the world’s largest populations with PG. Beside the well-known association with chronic bowel diseases and neoplasms, a potentially relevant new aspect is an association with endocrine diseases, in particular the metabolic syndrome, thyroid dysfunctions and renal disorders. Our findings represent clinically relevant new aspects. This may help to describe the patients’ characteristics and help to understand the underlying pathophysiology in these often misdiagnosed patients.

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          Most cited references27

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          Psoriasis and systemic inflammatory diseases: potential mechanistic links between skin disease and co-morbid conditions.

          Psoriasis is now classified as an immune-mediated inflammatory disease (IMID) of the skin. It is being recognized that patients with various IMIDs, including psoriasis, are at higher risk of developing "systemic" co-morbidities, e.g., cardiovascular disease (CVD), metabolic syndrome, and overt diabetes. In non-psoriatic individuals, the pathophysiology of obesity, aberrant adipocyte metabolism, diabetes, and CVDs involves immune-mediated or inflammatory pathways. IMIDs may impact these co-morbid conditions through shared genetic risks, common environmental factors, or common inflammatory pathways that are co-expressed in IMIDs and target organs. Given that pathogenic immune pathways in psoriasis are now well worked out and a large number of inflammatory mediators have been identified in skin lesions, in this review we will consider possible mechanistic links between skin inflammation and increased risks of (1) obesity or metabolic alterations and (2) CVD. In particular, we will discuss how well-established risk factors for CVD can originate from inflammation in other tissues.
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            Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria.

            Pyoderma gangrenosum is a rare but significant cause of ulcerations. It is a diagnosis of exclusion. Herein, we suggest diagnostic criteria and some historical perspectives on the diagnosis of pyoderma gangrenosum.
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              Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients.

              Pyoderma gangrenosum (PG) is an uncommon and challenging disease, highly associated with comorbidities, but poorly characterized from a diagnostic and therapeutic perspective. To describe the epidemiology of PG in a hospital-based retrospective review, focusing on demographics, comorbidities and treatments. We conducted a retrospective chart review. Patient data were taken from the Research Patient Data Repository of Brigham and Women's Hospital and Massachusetts General Hospital from 1 January 2000 to 31 December 2007. We identified and confirmed 103 cases of PG, and collected data on anatomical location, number and size of the PG lesions, patient demographics, comorbidities, mortality rate and treatments. Of the 103 patients, 78 (76%) were female, and only 7% had a biopsy suggestive of PG. The lower leg was the most common location with 78% of PG ulcers occurring there, and 67 (65% of patients) had two or more ulcers at some point. Thirty-five individuals (34%) had inflammatory bowel disease (IBD), 21 (20%) had haematological disorders, 14 (14%) had major depression, 20 (19%) had seronegative arthritis, 11 (11%) had psoriasis, and nine (9%) had hepatitis. Therapy was generally multimodal. The mortality rate during the 8-year study period was 16%. We present one of the largest PG case series to date. In our study, we found that biopsy of a PG lesion rarely yielded characteristic features of the disease and tissue pathology should not be used to exclude a PG diagnosis. We also found a female predominance and associations with IBD and haematological disorders. Patients with PG in this series had high rates of depression and hepatitis. Further work is needed to establish the mechanism(s) underlying these findings. © 2011 The Authors. BJD © 2011 British Association of Dermatologists.
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                Author and article information

                Contributors
                Journal
                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central
                1750-1172
                2013
                8 September 2013
                : 8
                : 136
                Affiliations
                [1 ]Department of Dermatology and Allergy, Skin Cancer Center Hannover, Hannover Medical School, Hannover, Germany
                [2 ]Institute for Health Services Research in Dermatology and Nursing, University of Hamburg, Hamburg, Germany
                [3 ]Department of Dermatology, St. Barbara Clinics, Duisburg, Germany
                [4 ]Department of Dermatology, University of Tuebingen, Tuebingen, Germany
                [5 ]Department of Dermatology, Heinrich Heine University, Duesseldorf, Germany
                [6 ]Department of Dermatology, University of Muenster, Muenster, Germany
                [7 ]Praxis für Venen- und Hauterkrankungen, Jena, Germany
                [8 ]Department of Dermatology, University of Bonn, Bonn, Germany
                [9 ]Department of Dermatology, Regensburg University Hospital, Regensburg, Germany
                [10 ]Department of Dermatology, Venereology and Allergology, University Hospital of Leipzig, Leipzig, Germany
                [11 ]Department of Dermatology, General Hospital of Goerlitz, Goerlitz, Germany
                [12 ]Department of Dermatology, General Hospital of Krefeld, Krefeld, Germany
                [13 ]Department of Dermatology and Allergology, University Hospital of Ulm, Ulm, Germany
                [14 ]Department of Dermatology, University of Cologne, Cologne, Germany
                [15 ]Hospital Dresden-Friedrichstadt, Academic Teaching Hospital of the Technical University of Dresden, Dresden, Germany
                [16 ]Department of Dermatology and Allergology, Hospital of Bremen-Mitte, Bremen, Germany
                [17 ]Department of Dermatology and Allergology, Ludwig-Maximilians-University Munich, Munich, Germany
                [18 ]Departement of Dermatology, Vital Clinic, Alzenau, Germany
                [19 ]Department of Dermatology, Hospital of Luedenscheid, Luedenscheid, Germany
                [20 ]Department of Dermatology, Venereology and Allergology, University School of Medicine Essen-Duisburg, Hufelandstrasse 55, Essen 45122, Germany
                Article
                1750-1172-8-136
                10.1186/1750-1172-8-136
                3844435
                24010984
                aec079ae-534e-4933-b711-d28d0bddea9d
                Copyright © 2013 Al Ghazal et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 20 June 2013
                : 30 August 2013
                Categories
                Research

                Infectious disease & Microbiology
                pyoderma gangrenosum,chronic ulcer,comorbidities,metabolic syndrome,diabetes mellitus

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