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      A Rare Case of Pyoderma Gangrenosum in a Patient With Pancreatic Neuroendocrine Tumor

      case-report
      Author(s): 1 ,
      Editor(s): ,
      Publication date (Electronic, pub):
      Publication date (Electronic, collection):
      Journal: Cureus
      Publisher: Cureus
      Keywords: pyoderma gangrenosum

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          Abstract

          Pyoderma gangrenosum is a rare inflammatory skin disease usually associated with an underlying internal condition. Diseases most commonly linked with pyoderma gangrenosum are inflammatory bowel disease (ulcerative colitis and Crohn’s disease), rheumatoid arthritis, and hematologic malignancies such as leukemia and lymphoma. In rare instances, it can occur as a type of paraneoplastic syndrome in patients with cancer. The most common cancers associated with pyoderma gangrenosum are breast cancer followed by rectal, gastric, renal, and lung cancers. There are scarce reports of neuroendocrine tumors associated with pyoderma gangrenosum. We discuss a case of a pancreatic neuroendocrine cancer patient afflicted with pyoderma gangrenosum.

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          Pyoderma gangrenosum: an updated review.

          Jason Miranda, A Gravina, E Ruocco … (2009)
          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.
          Article 0 comments Cited 137 times – based on 0 reviews     Review now
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            Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment.

            Afsaneh Alavi, Lars E. French, Mark D M Davis … (2017)
            Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic disorder with prototypical clinical presentations. Its pathophysiology is complex and not fully explained. Recent information regarding the genetic basis of PG and the role of auto-inflammation provides a better understanding of the disease and new therapeutic targets. PG equally affects patients of both sexes and of any age. Uncontrolled cutaneous neutrophilic inflammation is the cornerstone in a genetically predisposed individual. Multimodality management is often required to reduce inflammation, optimize wound healing, and treat underlying disease. A gold standard for the management of PG does not exist and high-level evidence is limited. Multiple factors must be taken into account when deciding on the optimum treatment for individual patients: location, number and size of lesion/ulceration(s), extracutaneous involvement, presence of associated disease, cost, and side effects of treatment, as well as patient comorbidities and preferences. Refractory and rapidly progressive cases require early initiation of systemic therapy. Newer targeted therapies represent a promising pathway for the management of PG, and the main focus of this review is the management and evidence supporting the role of new targeted therapies in PG.
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              Pyoderma gangrenosum - a guide to diagnosis and management .

              Christina George, Florence Deroide, Malcolm Rustin (2019)
              Pyoderma gangrenosum (PG) is a reactive non-infectious inflammatory dermatosis falling under the spectrum of the neutrophilic dermatoses. There are several subtypes, with 'classical PG' as the most common form in approximately 85% cases. This presents as an extremely painful erythematous lesion which rapidly progresses to a blistered or necrotic ulcer. There is often a ragged undermined edge with a violaceous/erythematous border. The lower legs are most frequently affected although PG can present at any body site. Other subtypes include bullous, vegetative, pustular, peristomal and superficial granulomatous variants. The differential diagnosis includes all other causes of cutaneous ulceration as there are no definitive laboratory or histopathological criteria for PG. Underlying systemic conditions are found in up to 50% of cases and thus clinicians should investigate thoroughly for such conditions once a diagnosis of PG has been made. Treatment of PG remains largely anecdotal, with no national or international guidelines, and is selected according to severity and rate of progression. Despite being a well-recognised condition, there is often a failure to make an early diagnosis of PG. This diagnosis should be actively considered when assessing ulcers, as prompt treatment may avoid the complications of prolonged systemic therapy, delayed wound healing and scarring.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 28 December 2020
                Publication date (Electronic, collection): December 2020
                Volume: 12
                Issue: 12
                Electronic Location Identifier: e12343
                Affiliations
                [1 ] Internal Medicine, Moffitt Cancer Center, Tampa, USA
                Author notes
                Article
                DOI: 10.7759/cureus.12343
                PMC ID: 7837650
                PubMed ID: 33520538
                SO-VID: 386791df-cf61-4b41-9af6-5deb8c9e1394
                Copyright © Copyright © 2020, Wesolow et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date accepted : 28 December 2020
                Categories
                Subject: Dermatology
                Subject: Internal Medicine
                Subject: Oncology

                Keywords: pyoderma gangrenosum
                Data availability:
                Keywords: pyoderma gangrenosum

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