Pyoderma Gangrenosum Associated With Inflammatory Bowel Disease. Report of Two Cases With Good Response to Infliximab

Servicio de Reumatología, Hospital de Jerez de Frontera, Cádiz, Spain.
Reumatología clinica 11/2011; 8(2):90-2. DOI: 10.1016/j.reumae.2011.07.006
Source: PubMed


Among the extraintestinal manifestations of inflammatory bowel disease (IBD), pyoderma gangrenosum (PG) often poses a therapeutic challenge. We describe two cases of PG associated with inflammatory bowel disease, who responded to treatment with Infliximab.

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    ABSTRACT: Pyoderma gangrenosum (PG) in inflammatory bowel disease (IBD) is uncommon and therapeutically challenging. Its treatment remains poorly characterised due to limited individual centre or practitioner experience. No large series are reported since 2003, yet IBD treatment has changed substantially. To provide an up-to-date review of the published treatment efficacy of currently available therapies for IBD-related PG in the biologic era. Systematic review of cases published post-2003 since the broad availability of anti-tumour necrosis factor-alpha (TNFα) therapy. Cases which did not have coexistent IBD, were non-English language, of paediatric age or without data on response to therapy were excluded. Sixty cases were identified; 55% female, 50% UC, 45% CD, 5% IBD-U. At PG diagnosis, 58% had active and only 15% inactive IBD, with 27% with IBD activity unspecified. Predominant sites were lower limb (48%) and peristomally (25%); 42% had multiple lesions. In 12%, trauma preceded PG. In 42%, new PG appeared whilst on IBD-specific therapy, whilst 28% were on no therapy and in 30%, IBD therapy was unspecified. Of patients on no therapy at PG onset (n = 17), 16 healed; seven with first- and eight with second-line therapy. In total, 34/60 patients received infliximab, four received adalimumab, two had both; with 33 (92%) responding to one or the other. There was no correlation of PG duration or size with healing times. Pyoderma gangrenosum appears predominantly during active IBD and is seen equally in CD and UC. New PG may be a manifestation of recrudescent IBD or it follow trauma. Anti-TNFα therapy as a first-line agent for PG should be considered, as it appears to be highly effective.
    Full-text · Article · Aug 2013 · Alimentary Pharmacology & Therapeutics
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    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.
    No preview · Article · Oct 2013 · Inflammatory Bowel Diseases
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    ABSTRACT: Background Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful skin ulcerations for which treatment can be challenging. The genetic basis of pyoderma gangrenosum may provide a better understanding of the disease and new targets for treatment.Objectives The purpose of this study is to systematically review the published literature regarding the syndromes and genetic mutations associated with PG.MethodsA literature search was performed through clinical queries PubMed (National Library of Medicine) database and the Cochrane database. The studies were assessed and then categorized as relating to syndromes or specific gene mutations.Results208 articles were identified, describing 823 cases of pyoderma gangrenosum. 537 cases (65.2%) were associated with inflammatory bowel disease (IBD), 133 cases (16.1%) were associated with polyarthritis, and 103 (12.5%) with hematologic disorders. 31 cases of PAPA and its variants were identified. Two patients had MTHFR mutations and two had JAK2 gene mutations. Fourteen (1.7%) were familial cases. PG responded to different treatments depending on the setting. For example, treatment with B vitamins improved PG in cases of MTHFR mutations, whereas patients with myelodysplastic syndrome improved with thalidomide treatment.Conclusions Pyoderma gangrenosum can occur in isolation, associated with systemic disease, or as part of various syndromes. Different genetic causes may be best treated with particular treatments. Understanding its genetic basis can help elucidate new potential targets for drug development.This article is protected by copyright. All rights reserved.
    No preview · Article · Oct 2014 · British Journal of Dermatology