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Classification of Crohn's disease patients (n=1015)*
Source publication
Erythema nodosum and pyoderma gangrenosum may occur in Crohn's disease. In the present evaluation of consecutive patients with Crohn's disease spanning more than two decades, erythema nodosum was seen in 45 patients and pyoderma gangrenosum was seen in seven patients. Forty-one of 566 women (7.2%) and nine of 449 men (2.0%) were affected. Of these,...
Contexts in source publication
Context 1
... 1 shows that this population was predomi- nantly female with over 80% diagnosed before 40 years of age, similar to a previous report for the initial 877 patients (1). As shown in Table 1, disease was most often detected in the ileocolon with a high rate of complex disease (ie, over 70%), including stric- ture formation and penetrating complications. As noted elsewhere (1-3), for all patients in the present evaluation, office and inpa- tient hospitalization records as well as endoscopic, radiological imaging, surgical and pathology reports were recorded. ...
Context 2
... others in this population with pyoderma gangrenosum, specifi- cally, three women and three men, were each diagnosed with Crohn's disease later, between 20 and 40 years of age. Table 1 shows the percentage of patients in each age group based on disease location as defined by the modern Vienna schema (1), but modified to permit evaluation of patients diag- nosed younger than 20 years of age (3). As shown, patients diagnosed younger than 20 years of age had more extensive dis- ease, usually with ileocolonic involvement and disease in the upper gastrointestinal tract (P<0.05) while patients diagnosed older than 40 years of age more often had disease localized to the colon alone (P<0.05). ...
Context 3
... date, no colonic disease has been detected. Table 1 shows the percentages of patients in this population classified with different types of disease behaviour. For each of the different age groups, most patients suffered from complex clinical disease with strictures or penetrating complications. ...
Similar publications
We reported the successful treatment with oral methylprednisolone and cyclosporine in combination with topical wound care on a boy with pyoderma gangrenosum presenting as huge and deep ulceration on buttocks and legs. We reported the successful treatment with oral methylprednisolone and cyclosporine in combination with topical wound care on a boy w...
Citations
... PG is the most damaging of the cutaneous extraintestinal manifestations of IBD and leads to repeated hospitalizations, increased morbidity (hazard ratio=1.72) and even mortality (54)(55)(56)(57)(58)(59). Treatment strategies are inadequate, and traditional therapies employed to manage PG can aggravate intestinal inflammation and vice versa. ...
Pyoderma gangrenosum (PG) is a debilitating skin condition often accompanied by inflammatory bowel disease (IBD). Strikingly, ~40% of patients that present with PG have underlying IBD, suggesting shared but unknown mechanisms of pathogenesis. Impeding the development of effective treatments for PG is the absence of an animal model that exhibits features of both skin and gut manifestations. This study describes the development of the first experimental drug-induced mouse model of PG with concomitant intestinal inflammation. Topical application of pyrimidine synthesis inhibitors on wounded mouse skin generates skin ulcers enriched in neutrophil extracellular traps (NETs) as well as pro-inflammatory cellular and soluble mediators mimicking human PG. The mice also develop spontaneous intestinal inflammation demonstrated by histologic damage. Further investigations revealed increased circulating low density IL-1β primed neutrophils that undergo enhanced NETosis at inflamed tissue sites supported by an increase in circulatory citrullinated histone 3, a marker of aberrant NET formation. Granulocyte depletion dampens the intestinal inflammation in this model, further supporting the notion that granulocytes contribute to the skin-gut crosstalk in PG mice. We anticipate that this novel murine PG model will enable researchers to probe common disease mechanisms and identify more effective targets for treatment for PG patients with IBD.
... Lesions range in size from 1 to 5 cm in diameter. EN can develop in numerous areas at the same time, and studies have estimated that the incidence of recurring illness is around 20%. 46 However, the prevalence of this disorder is comparatively higher in individuals with CD as compared to those with UC. Lesions appear in tandem with intestinal disease activity, and lesions generally disappear as bowel illness declines; hence, therapy is typically directed at the underlying disease affecting the bowel. ...
The prevalence and incidence of Ulcerative Colitis (UC), a recurrent and remitting inflammatory condition, are rising. Any part of the colon may be affected, beginning with inflammation of the mucosa in the rectum and continuing proximally continuously. Bloody diarrhea, tenesmus, fecal urgency, and stomach pain are typical presenting symptoms. Many patients present with extraintestinal manifestations (EIMs) including musculoskeletal, ocular, renal, hepatobiliary, and dermatological presentation, among others. Most cases are treated with pharmacological therapy including mesalazine and glucocorticoids. Fecal microbiota transplantation (FMT) is a novel procedure that is increasingly being used to treat UC, however, its use yet remains controversial because of uncertain efficacy. FMT can lower gut permeability and consequently disease severity by boosting short-chain fatty acids production, helping in epithelial barrier integrity preservation. Upadacitinib (JAK Kinase inhibitor) is another newer treatment option, which is an FDA-approved drug that is being used to treat UC. This review article provides a comprehensive review of the EIMs of UC, the role of FMT along with various recent clinical trials pertaining to FMT as well as other diagnostic and therapeutic updates.
... PG can occur in parallel with IBD activity or take an independent course. PG tends to recur following successful treatment in more than 25% of cases, often in the same place as the initial episode [55]. The lesions initially appear as single or multiple erythematous papules or pustules that are often preceded by trauma to the skin (pathergy).They occur most commonly on the lower legs and peristomal areas in up to 80% and 18% of cases, respectively; however, PG can appear anywhere on the body surface [55]. ...
... PG tends to recur following successful treatment in more than 25% of cases, often in the same place as the initial episode [55]. The lesions initially appear as single or multiple erythematous papules or pustules that are often preceded by trauma to the skin (pathergy).They occur most commonly on the lower legs and peristomal areas in up to 80% and 18% of cases, respectively; however, PG can appear anywhere on the body surface [55]. Subsequent necrosis of the dermis leads to the development of deep ulcerations that contain purulent material that is usually sterile on culture [59]. ...
Crohn’s disease (CD) and ulcerative rectocolitis (UC) are chronic and recurrent inflammatory bowel diseases, with a broad therapeutic arsenal for the treatment of intestinal disease. In addition to intestinal involvement, about one third of patients have extra-intestinal manifestations (EIMs), associated with important morbidity and complications, sometimes occurring independently of the inflammatory activity of intestinal disease. Due to their complexity, EIMs require adequate systematization for diagnosis, treatment and follow-up, involving different specialties. The limited number of clinical trials for some of these manifestations adds to the challenges. In this review, we discuss the most frequent and relevant EIMs of IBD by highlighting the epidemiological, clinical, and diagnostic aspects and the main available therapeutic options.
... Face, trunk, and upper extremities are seldom affected. EN can simultaneously involve multiple locations and its recurrence rate is approximately 20% [33]. Of note, EN lesions correlate with the underlying disease activity and worsen with colitis flares in IBD patients [29], but not with the disease severity/extent. ...
... Skin biopsy is generally not necessary, as the diagnosis of EN is often based on the Face, trunk, and upper extremities are seldom affected. EN can simultaneously involve multiple locations and its recurrence rate is approximately 20% [33]. Of note, EN lesions correlate with the underlying disease activity and worsen with colitis flares in IBD patients [29], but not with the disease severity/extent. ...
Inflammatory bowel diseases (IBDs) may be associated with extra-intestinal manifestations. Among these, mucocutaneous manifestations are relatively frequent, often difficult to diagnose and treat, and may complicate the course of the underlying disease. In the present review, a summary of the most relevant literature on the dermatologic manifestations occurring in patients with inflammatory bowel diseases has been reviewed. The following dermatological manifestations associated with IBDs have been identified: (i) specific manifestations with the same histological features of the underlying IBD (occurring only in Crohn’s disease); (ii) cutaneous disorders associated with IBDs (such as aphthous stomatitis, erythema nodosum, psoriasis, epidermolysis bullosa acquisita); (iii) reactive mucocutaneous manifestations of IBDs (such as pyoderma gangrenosum, Sweet’s syndrome, bowel-associated dermatosis-arthritis syndrome, aseptic abscess ulcers, pyodermatitis–pyostomatitis vegetans, etc.); (iv) mucocutaneous conditions secondary to treatment (including injection site reactions, infusion reactions, paradoxical reactions, eczematous and psoriasis-like reactions, cutaneous infections, and cutaneous malignancies); (v) manifestations due to nutritional malabsorption (such as stomatitis, glossitis, angular cheilitis, pellagra, scurvy, purpura, acrodermatitis enteropathica, phrynoderma, seborrheic-type dermatitis, hair and nail abnormalities). An accurate dermatological examination is essential in all IBD patients, especially in candidates to biologic therapies, in whom drug-induced cutaneous reactions may assume marked clinical relevance.
... This can be treated by removing the metastatic CD. EN is one the most observed dermatological manifestation in individuals with IBD and is high frequent in women and CD patients (4%-15% CD vs. 3%-10% CD cases) 65, 66 . EN is linked with active IBD in general, however not by its severity 67 . ...
Inflammatory bowel disease (IBD) encompasses a spectrum of diseases, with Crohn's disease (CD) and ulcerative colitis (UC)
representing the two broadest subtypes of IBD. Multiple extraintestinal manifestations (EIMs) are more frequent in (IBD); 5% –
50% of the patients might be affected. The most often implicated sites of manifestations are musculoskeletal and dermatological
structures. However, while some symptoms like peripheral arthritis and erythema nodosum correlate with IBD progression, others
have their own course of disease like axial arthropathy, gangrenosis of the pioderma and primary sclerosic cholangitis. This review
would provide a summary of the most frequent EIMs and their prevalence.
Keywords: Crohn's disease, inflammatory bowel disease, Ulcerative colitis.
... This can be treated by removing the metastatic CD. EN is one the most observed dermatological manifestation in individuals with IBD and is high frequent in women and CD patients (4%-15% CD vs. 3%-10% CD cases) 65, 66 . EN is linked with active IBD in general, however not by its severity 67 . ...
Inflammatory bowel disease (IBD) encompasses a spectrum of diseases, with Crohn's disease (CD) and ulcerative colitis (UC) representing the two broadest subtypes of IBD. Multiple extraintestinal manifestations (EIMs) are more frequent in (IBD); 5% –50% of the patients might be affected. The most often implicated sites of manifestations are musculoskeletal and dermatological structures. However, while some symptoms like peripheral arthritis and erythema nodosum correlate with IBD progression, others have their own course of disease like axial arthropathy, gangrenosis of the pioderma and primary sclerosic cholangitis. This review would provide a summary of the most frequent EIMs and their prevalence.
Keywords: Crohn's disease, inflammatory bowel disease, Ulcerative colitis.
... This can be treated by removing the metastatic CD. EN is one the most observed dermatological manifestation in individuals with IBD and is high frequent in women and CD patients (4%-15% CD vs. 3%-10% CD cases) 65, 66 . EN is linked with active IBD in general, however not by its severity 67 . ...
... Между ПГ и активностью ВЗК существует обратная взаимосвязь, так как ПГ может протекать паралельно обострению ВЗК или независимо. Также ПГ имеет тенденцию рецидивировать после успешного лечения более чем в 25% случаев, часто в том же месте, где возникала впервые [47]. Терапия ГП включает применение системных ГКС, инфликсимаба или адалимумаба либо топических или пероральных ингибиторов кальциневрина [48]. ...
The article is devoted to the current data regarding the pathogenesis, classification and frequency of extraintestinal manifestations (EIMs) in inflammatory bowel diseases. We discuss two distinct theories of EIMs pathogenesis. First, EIMs arise from an extension of antigen-specific immune responses from the intestine to non-intestinal sites. Second, EIMs are independent inflammatory events initiated or perpetuated by the presence of IBD or by shared genetic or environmental risk factors in the host. These mechanisms are not mutually exclusive and may contribute to varying degrees in different EIMs. Early diagnosis of EIMs contributes to prevention disability and enhancement of quality of life of IBD patients. It is concluded that treatment of extraintestinal manifestations should be carried out taking into account the course of the IBD and the multidisciplinary approach, which requires close cooperation of doctors of various specialties. Assessment of prognostic markers and predictors for EIM in IBD will be part of a future investigation.
... Our study also confirmed the tendency for this condition to be more common in CD patients and for there to be a higher prevalence in women. Pyoderma gangrenosum is a much rarer, more severe, and debilitating EIM that is more common in UC patients than in CD patients [37][38][39]. Its prevalence has been reported to be 1%-10% in UC patients and 0.5%-20% in CD patients [40]. ...
Background
The prevalence of inflammatory bowel disease (IBD) in South Korea is increasing. Although extraintestinal manifestations (EIMs) are an important factor in the clinical outcomes of IBD patients, EIMs have not yet been investigated in Korea. Thus, we conducted a cross-sectional study to assess the prevalence of EIMs in Korean IBD patients.
Methods
The 2014 claims data from the National Health Insurance System (NHIS) of Korea were used. IBD patients were identified by codes for Crohn disease (CD) and ulcerative colitis (UC) in the NHIS registration system for rare or intractable diseases. International Classification of Diseases, Tenth Edition codes were used to identify EIM cases. To estimate the prevalence of EIMs in the general population of Korea, we used national sample data. Standardized prevalence ratios (SPRs) were calculated to compare the prevalence rates of EIMs among IBD patients to those among the general population of Korea.
Results
A total of 13,925 CD patients and 29,356 UC patients were identified. CD and UC patients were different in terms of demographics and utilization of medication. Among the 17 EIMs investigated, pyoderma gangrenosum, osteomalacia, Sweet syndrome, and scleritis were observed in very few patients. The SPRs were greater than 1 for all EIMs. Aphthous stomatitis, rheumatoid arthritis, and osteoporosis were highly prevalent in both CD and UC patients, but the SPRs of the EIMs were not high.
Conclusion
The study confirmed that EIMs are more prevalent among IBD patients than among the general population of Korea. The prevalence of EIMs in IBD patients suggests the need for greater attention and effort in clinical practice.
... We noted a mean age of first episode PG onset of 39 years. This age is somewhat younger compared to other series in which the age of PG diagnosis has ranged from 45 to 62 [23][24][25][26][27] . However, these published cases were not necessarily limited to patients with IBD and so included patients with PG associated with hematologic disorders, for example, more commonly seen in older individuals. ...
... Interestingly, 13% of patients were already on an anti-TNF at the time of PG onset. This may suggest a unique mechanism of PG development in this subset of patients given that anti-TNF has been demonstrated to heal PG in a small randomized control trial and a number of small case series [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32] . A similar parallel has been noted with psoriasis, a dermatologic lesion also treated with anti-TNF therapy. ...
Pyoderma gangrenosum (PG) is a severe extraintestinal manifestation of inflammatory bowel disease (IBD).
To better characterize PG features and management among an IBD cohort.
Subjects with PG were identified using a large database at a tertiary center. Patient demographics and clinical characteristics were summarized using descriptive statistics.
Eighty patients with an episode(s) of PG were identified, yielding an overall prevalence of 1.9%. Overall, 93% of patients with PG had some degree of colonic inflammation. Thirty-one (39%) patients required hospitalization for PG. Underlying bowel disease was active at the time of PG episode(s) in 52 (65%) patients. The PG location was variable, with the lower extremity being the most common. Most patients (71.3%) required multiple therapies to achieve PG healing.
We describe one of the largest case series of PG among patients with IBD. The variety of treatment strategies used highlights the lack of clear guidelines in managing this complex group of patients.
© 2014 Canadian Dermatology Association.
