Bowel-associated Dermatosis-arthritis Syndrome Research Articles

BIOLOGICS AND SMALL MOLECULES USED IN IBD MIMICS: HIGHLIGHTING THE IMPORTANCE OF DIAGNOSIS CONFIRMATION

Abstract INTRODUCTION Since 2009, inflammatory bowel disease (IBD) specialists have been utilizing “IBD Live,” a weekly live video conference with a global audience of 150-200 participants, to discuss the multidisciplinary management of their commonly seen and most challenging cases. While most cases presented were confirmed as IBD, a substantial number were mimics for which IBD was not the ultimate diagnosis. We previously categorized all IBD Live cases and identified the frequency of “IBD mimics.” Here, we examine the use of biologic medications for these cases which ultimately were determined to be something other than IBD. METHODS Cases have been recorded and archived since May 2018. 371 total cases from May 2018 through February 2023 were reviewed spanning 186 hours. 65 of those cases were determined to be IBD mimics, defined as those with features of IBD that ultimately resulted in a non-IBD diagnosis. IBD mimics were analysed and categorized in terms of their biologic usage. RESULTS 25 of the 65 mimics (38.5%) were treated with biologics for presumed IBD. 14 were treated with one biologic, 5 with two biologics, and 6 with three or more biologics. The biologics used in the management of these IBD mimics included infliximab (n=21), vedolizumab (n=12), adalimumab (n=11), ustekinumab (n=6), and certolizumab (n=1). Of the 25 cases of biologic usage without definitive confirmation of IBD, 6 ended up with a diagnosis where biologics were an appropriate treatment for the mimic. These mimics included drug-induced colitis (n=3), Behcet’s disease (n=1), segmental colitis associated with diverticulosis (SCAD; n=1), and CTLA-4 haploinsufficiency with autoimmune infiltrates (CHAI; n=1). Several of the IBD mimics were prescribed steroids, azathioprine, mercaptopurine, or methotrexate, and 3 were trialled on tofacitinib (for Sweet’s syndrome, PIK3cd, and SCAD). There were 3 patients with confirmed IBD, in remission on a biologic, who subsequently had symptoms concerning for an IBD flare. In each of these cases, IBD was not the cause of their symptoms (e.g., cecal volvulus). There were 4 patients with confirmed IBD, in remission off biologics, who later developed symptoms suspicious for an IBD flare. Each of these cases was trialled on biologics to treat the supposed flare; however, symptoms were ultimately found to be due to an IBD mimic (e.g., eosinophilic esophagitis). 33 of the 65 IBD mimics were never treated with biologics. DISCUSSION In a 5-year period at IBD Live, 17.5% of cases (65 of 371) were found to be IBD mimics. 38.5% (25 of 65) of these were treated with biologics. The diverse presentation of signs and symptoms of IBD and IBD mimics requires reassessment of the biologic therapy in patients without response. Often the biologic needs to be optimized for IBD, but in some cases, the diagnosis of IBD will need to be called into question. Table 1 IBD mimics and biologics used Abbreviations BADAS = bowel-associated dermatosis-arthritis syndrome, BCL = B-cell lymphoma, CD = Crohn’s Disease, CHAI = CTLA-4 haploinsufficiency with autoimmune infiltrates, C-MUSE = cryptogenic multifocal ulcerous stenosing enteritis, CMV = cytomegalovirus, CVID = common variable immunodeficiency, EGPA = eosinophilic granulomatosis with polyangiitis, GABA = gamma-aminobutyric acid, IBD = inflammatory bowel disease, IMHMV = idiopathic myointimal hyperplasia of the mesenteric veins, PIK3cd = phosphatidylinositol-4,5-bisphosphonate 3-kinase catalytic subunit delta, PNH = paroxysmal nocturnal hemoglobinuria, SCAD = segmental colitis associated with diverticulosis, STI = sexually transmitted infection, UC = Ulcerative Colitis. Image 1: Biologic usage in IBD mimics

44505 Bowel-associated dermatosis-arthritis syndrome masquerading as viral rash: Recognizing a rare cutaneous manifestation of inflammatory bowel disease

44505 Bowel-associated dermatosis-arthritis syndrome masquerading as viral rash: Recognizing a rare cutaneous manifestation of inflammatory bowel disease

Ulcerative colitis and pyoderma gangrenosum presentation of a case and brief literature review

Inflammatory bowel disease (IBD) is a chronic immune-mediated disorder comprised of Crohn's disease and ulcerative colitis. Ulcerative colitis affects the large intestine, while Crohn's disease can affect any part of the gastrointestinal tract (GIT). IBD is a multisystem condition that predominantly affects the gastrointestinal, musculoskeletal, ocular, and cutaneous systems. The following dermatological manifestations associated with IBDs have been identified: Specific manifestations with the same histological features of the underlying only ocurrs in Crohn Disease (CD); cutaneous disorders associated with IBD aphthous stomatitis, erythema nodosum; reactive mucocutaneous manifestations of IBDs pyoderma gangrenosum, Sweet’s syndrome, bowel-associated dermatosis-arthritis syndrome, aseptic abscess ulcers, pyodermatitis y mucocutaneous conditions secondary to treatment including injection site reactions y eczema and manifestations due to nutritional malabsorption such as stomatitis, glossitis, angular cheilitis hair and nail abnormalities. We believe 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, such as psoriasiform eruption. One of the cutaneous manifestations associated with UC is pyoderma gangrenosum (PG), which is an inflammatory neutrophilic dermatosis that clinically presents with well-defined ulcers with an erythematous border and mucopurulent or hemorrhagic exudate.We present the case of a 44-year-old female patient, who presented non-specific chronic ulcerative colitis (UC) with active intestinal symptoms, fever, diarrhea and hematochezia, 20 days later it began with pustules on the head, trunk and extremities, which evolved into an ulcer with a border erythematous and well defined whose clinical and histopathological diagnosis corresponded to pyoderma gangrenosum.

Bowel-associated dermatosis-arthritis syndrome (BADAS): a narrative review.

Bowel-associated dermatosis-arthritis syndrome is a neutrophilic dermatosis presenting with flu-like symptoms, arthritis and skin lesions. The type of skin presentation varies although typically consists of red patches progressing into papulopustular-type lesions. Initially, the pathogenesis of the disease was linked exclusively to bariatric surgery, but more recent reports indicate the role of gastrointestinal diseases resulting in bacterial overgrowth and consequent translocation of bacteria into the bloodstream. The disease may resolve spontaneously, however sometimes requires a treatment aimed at managing inflammation and excessive bacterial overgrowth, and therefore antibiotic therapy, immunosuppressive drugs, biological treatment and sometimes intestinal surgeries are applied.

Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis

article: Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis - Italian Journal of Dermatology and Venereology 2021 December;156(Suppl. 1 to No. 6):7-8 - Minerva Medica - Journals

Novel Therapeutic Approaches and Targets for the Treatment of Neutrophilic Dermatoses, Management of Patients with Neutrophilic Dermatoses and Future Directions in the Era of Biologic Treatment.

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders characterized by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. Universally accepted and validated guidelines for the management of neutrophilic dermatoses do not exist, also given the paucity of randomized controlled study and high-quality data. However, the literature on the effective use of biologic therapies is rapidly expanding. This article reviews the epidemiology, clinical characteristics, histopathologic features, and management of pyoderma gangrenosum as well as Sweet's syndrome, sub-corneal pustular dermatoses and bowel-associated dermatosis arthritis syndrome. The use of biologic agents, including tumor necrosis factor α-inhibitors, anti-IL1, anti-IL-17, and IL-23 are discussed in detail.

Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis

article: Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis - Giornale Italiano di Dermatologia e Venereologia 2019 Mar 29 - Minerva Medica - Journals

Rare, but Serious Extra-intestinal Complication of Inflammatory Bowel Disease

Bowel-associated dermatosis-arthritis syndrome (BADAS) has been linked to Inflammatory Bowel Disease. Classic prodrome includes fever, chills, myalgias, and polyarthralgias that later evolve into inflammatory papules similar to Sweet Syndrome. The papules consist of neutrophilic infiltrate without vessel wall destruction, differentiating it from other neutrophilic dermatoses. We present a case of a patient with Ulcerative Colitis who also underwent gastric sleeve surgery and presented with BADAS. A 38 year-old Caucasian female presented with diffuse rash for one-week duration. Patient reported fever, chills and myalgias for two days. The rash, consisting of blisters and pustules, erupted initially on her face and spread to her trunk and extremities. She denied pruritus, but did report a burning and stinging sensation. Arthralgias were also noted in bilateral shoulders and knees. She denied any changes in bowel habits or recent illness. The patient had previously been on infliximab, but was discontinued approximately six months earlier. The patient was febrile (101.8F), tachycardic (113 bpm), and hypertensive (150/78 mmHg) on admission. She was in slight distress secondary to symptom burden. On examination, her skin demonstrated multiple pustules with peripheral erythema and crusting. These pustules were noted on her head, face, upper and lower extremities sparing the palms and soles. The rash was non-tender. On admission, her white blood cell count was 16.1 (TH/mm3) hemoglobin 13.1 (g/dL), platelets 341 (TH/mm3), total protein 7 (g/dL), AST 50 (U/L), ALT 62 (U/L), CRP 11.13 (mg/dL), ESR 60 (mm/hr). Her pregnancy, HIV, Hepatitis panel, direct streptococcal antigen, mononucleosis antibody, and mycoplasma IgM testing were all negative. Skin biopsy demonstrated mature neutrophilic infiltrate. This involved the dermis and caused papillary dermal edema. There was no noted fibrinoid necrosis or infarction of the vessels. This was consistent with diagnosis of BADAS given the patient's history of UC. The patient was treated with valacyclovir, steroids, anti-histamine agents and responded well. Our case demonstrates extra intestinal manifestation of IBD that is often not considered in differential diagnosis. Our goal is to bring awareness due to its common recurrence and prompt early treatment initiation for resolution.2048_A Figure 1. Abdomen, punch biopsy. Suppurative folliculitis with a ruptured follicle.2048_B Figure 2. Abdomen, punch biopsy demonstrating neutrophilic infiltrate. Suppurative folliculitis with a ruptured follicle.2048_C Figure 3. Abdomen, punch biopsy. Suppurative folliculitis with a ruptured follicle.

Bowel-Associated Dermatosis-Arthritis Syndrome (BADAS) as Initial Presentation of Crohnʼs Disease

Bowel-Associated Dermatosis-Arthritis Syndrome (BADAS) as Initial Presentation of Crohnʼs Disease

Neutrophilic dermatoses: Kids are not just little people

Neutrophilic dermatoses are a group of inflammatory skin disorders characterized by an overactive innate immune system with dysregulation of neutrophils without underlying infectious etiology. The major representative conditions discussed are Sweet syndrome; pyoderma gangrenosum; neutrophilic eccrine hidradenitis; palmoplantar eccrine hidradenitis; subcorneal pustular dermatoses; bowel-associated dermatosis arthritis syndrome; and synovitis, acne, pustulosis, hyperostosis, and osteitis. We will also discuss other neutrophilic conditions present almost exclusively in the pediatric population, including congenital erosive and vesicular dermatosis with reticulated supple scarring and the recently described group of autoinflammatory diseases. The clinical characteristics, diagnostic approach, and treatment management in the pediatric and adult population are discussed.

Cutaneous involvement in chronic inflammatory bowel disease : Crohn's disease and ulcerative colitis

Over recent decades, both the incidence and prevalence of chronic inflammatory bowel disease have continued to rise in industrialized countries; the disease is frequently associated with extracutaneous involvement and comorbidity. The purpose of this work was to investigate the frequency and specificity of mucocutaneous manifestations in Crohn's disease (CD) and ulcerative colitis (UC). An extensive search in peer-reviewed journals via PubMed was performed; presented is a summary and analysis of various studies and data, including data of patients treated at our department. CD and UC are frequently associated with mucocutaneous symptoms; however, primary/specific disease-associations are exclusively seen in CD patients. These include peri-anal and -stomal fistulas and ulcerations, "metastatic" Crohn's disease as well as oral granulomatous disease. Moreover, in both CD and UC, there occur several other inflammatory skin conditions such as erythema nodosum, pyoderma gangrenosum, hidradenitis suppurativa, chronic oral aphthous disease, Sweet syndrome, pyostomatitis vegetans, and bowel-associated dermatosis-arthritis syndrome. Malnutrition syndromes (zinc and vitamin deficiencies) are only rarely observed. On skin and oral/genital mucous membranes various different inflammatory manifestations may be observed during the course of CD or UC. However, most data about adirect pathogenic relationship of the gastrointestinal and dermatologic disorders are quite heterogeneous or even contradictory. Nevertheless, knowledge of these conditions and their possible association with CD and UC could be crucial for early diagnosis and initiation of an appropriate therapy and thus be essential to prevent secondary tissue damage.

Is it bowel-associated dermatosis-arthritis syndrome induced by small intestinal bacteria overgrowth?

IntroductionBowel-associated dermatosis-arthritis syndrome is rare systemic diseases characterized by a prodrome of fever, chills, and influenza-like symptoms with subsequent skin eruptions, myalgias, and polyarthralgias. It is reported to be occurred in Intestinal bypass surgery and inflammatory bowel disease.Case descriptionHerein, we described a 29-years-old man with Bowel-associated dermatosis-arthritis syndrome. He had no history of gastrointestinal surgery and inflammatory bowel disease. Distribution of the gut bacterial flora showed small intestinal bacterial overgrowth.Discussion and EvaluationIt is rarely form as Non-intestinal bypass road and non-inflammatory bowel disease was induced by small intestinal bacteria overgrowth.ConclusionsWe concluded that Immuno-inflammatory response to overgrowth of intestinal bacterial antigen induce the clinical symptoms of bowel-associated dermatosis-arthritis syndrome.

Bowel-associated dermatosis-arthritis syndrome clinically mimicking dermatomyositis as a manifestation of colon resection

Bowel-associated dermatosis-arthritis syndrome clinically mimicking dermatomyositis as a manifestation of colon resection

Syndrome arthro-cutané lors d’une rectocolite ulcéro-hémorragique : une manifestation extra-intestinale rare des maladies inflammatoires de l’intestin

Bowel-associated dermatosis-arthritis syndrome (BADAS) is characterized by combined pustular skin eruption and arthralgia. It may be associated with inflammatory bowel disease or bowel bypass surgery. We report a case of BADAS in a patient with ulcerative colitis. A 39-year-old woman was being treated for a severe flare-up of ulcerative colitis present over the preceding 2 months and treated with prednisone, azathioprine and cyclosporine. She was also presenting a cutaneous eruption and arthralgia that had begun three days earlier. Dermatological examination revealed profuse vesicular and pustular lesions. Biopsy specimens showed mature neutrophilic infiltrate within the dermis. A diagnosis of BADAS was made and the same treatment was maintained. Systemic symptoms were resolved but the vesicular lesions were superseded by hypertrophic scars. Bowel-associated dermatosis-arthritis syndrome consists of a vesiculopustular eruption associated with arthralgia and/or arthritis and fever, as was the case in our patient. The histological picture is characterized by abundant neutrophilic infiltrate in the superficial dermis. The clinical and histological features and the course of BADAS allow this entity to be classified within the spectrum of neutrophilic dermatoses. Treatment chiefly involves systemic corticosteroids.

Approach to and Management of the Neutrophilic Dermatoses

The neutrophilic dermatoses are a related set of disorders that share common features. These disorders are not directly related to infection and are considered forms of “sterile” neutrophilic inflammation. They can occur with an associated systemic disease or as a primary cutaneous process. Subcorneal pustular dermatosis, Sweet’s syndrome, erythema elevatum diutinum, bowel-associated dermatosis-arthritis syndrome, pyoderma gangrenosum, and neutrophilic eccrine hidradenitis represent the most common of these disorders. This review highlights the salient features of the neutrophilic dermatoses with an emphasis on clinical features, evaluation, and management.

Neutrophilic Dermatoses: An Update

Neutrophilic dermatoses constitute a heterogeneous group of dermatologic diseases, which are unified by the predominance of neutrophils within the inflammatory infiltrate on histopathology. The aims of this review were to provide an update on the clinical and histologic presentation of the main neutrophilic dermatoses and to develop a guide for clinical practice. A structured literature search of PubMed, Medline, and Embase was performed, using the key words "neutrophilic disorders", "cutaneous small vessel vasculitis", "Sweet's syndrome", "bowel associated dermatosis arthritis syndrome", "Behcet's", "palisaded neutrophilic and granulomatous dermatosis", "rheumatoid neutrophilic dermatitis", and "pyoderma gangrenosum". Related articles were screened for key terms and were included if appropriate. This group contains a wide spectrum of unique disorders, each with its own histologic and clinical subtleties, making specific diagnosis of a given entity within the group diagnostically challenging. The fact that overlapping forms of neutrophilic dermatoses, which share features of multiple neutrophilic dermatoses, are not uncommon makes the diagnoses more challenging.

Jejunoileal bypass as the main procedure in the onset of immune-related conditions: the model of BADAS

Bariatric surgery represents a common approach for the control of severe morbid obesity, reducing caloric intake by modifying the anatomy of the gastrointestinal tract. Following jejunoileal bypass, a large spectrum of complications has been described, with rheumatic manifestation present in up to 20% of cases. Although bowel bypass syndrome, also called blind loop syndrome, is a well-recognized complication of jejunoileal bypass, the same syndrome was recognized in patients who had not had intestinal bypass surgery, and the term the ‘bowel-associated dermatosis–arthritis syndrome’ (BADAS) was coined. The pathogenesis of BADAS is as yet poorly understood and only few data concerning this issue have been published in the literature. The aim of the present paper is to review the literature and to discuss putative pathogenic mechanisms of BADAS, focusing on the immune system.

Cutaneous manifestations of gastrointestinal disease

The gastrointestinal (GI) and cutaneous organ systems are closely linked. In part I of this continuing medical education article, the intricacies of this relationship were explored as they pertained to hereditary polyposis disorders, hamartomatous disorders, and paraneoplastic disease. Part II focuses on the cutaneous system's links to inflammatory bowel disease and vascular disorders. An in-depth analysis of inflammatory bowel disease skin findings is provided to aid dermatologists in recognizing and facilitating early consultation and intervention by gastroenterologists. Cutaneous signs of inflammatory bowel disease include fissures and fistulae, erythema nodosum, pyoderma gangrenosum, pyostomatitis vegetans, oral aphthous ulcers, cutaneous polyarteritis nodosa, necrotizing vasculitis, and epidermolysis bullosa acquisita. Additional immune-mediated conditions, such as diverticulitis, bowel-associated dermatosis-arthritis syndrome, Henoch-Schönlein purpura, dermatitis herpetiformis, and Degos disease, in which the skin and GI system are mutually involved, will also be discussed. Genodermatoses common to both the GI tract and the skin include Hermansky-Pudlak syndrome, pseudoxanthoma elasticum, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia, and blue rubber bleb nevus syndrome. Kaposi sarcoma is a neoplastic disease with lesions involving both the skin and the gastrointestinal tract. Acrodermatitis enteropathica, a condition of zinc deficiency, likewise affects both the GI and dermatologic systems. These conditions are reviewed with updates on the genetic basis, diagnostic and screening modalities, and therapeutic options. Finally, GI complications associated with vascular disorders will also be discussed.

Bowel-associated dermatosis-arthritis syndrome: another cutaneous manifestation of inflammatory intestinal disease

International Journal of DermatologyVolume 52, Issue 12 p. 1596-1598 Correspondence Bowel-associated dermatosis–arthritis syndrome: another cutaneous manifestation of inflammatory intestinal disease María Teresa Truchuelo MD , María Teresa Truchuelo MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorJavier Alcántara MD , Javier Alcántara MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorSergio Vano-Galván MD , Sergio Vano-Galván MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorPedro Jaén MD , Pedro Jaén MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorCarmen Moreno MD , Carmen Moreno MD Department of Dermatology University Hospital Ramón y Cajal Madrid Spain E-mail: [email protected]Search for more papers by this author María Teresa Truchuelo MD , María Teresa Truchuelo MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorJavier Alcántara MD , Javier Alcántara MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorSergio Vano-Galván MD , Sergio Vano-Galván MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorPedro Jaén MD , Pedro Jaén MD Department of Dermatopathology University Hospital Ramón y Cajal Madrid SpainSearch for more papers by this authorCarmen Moreno MD , Carmen Moreno MD Department of Dermatology University Hospital Ramón y Cajal Madrid Spain E-mail: [email protected]Search for more papers by this author First published: 29 May 2012 https://doi.org/10.1111/j.1365-4632.2011.05149.xCitations: 15Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Citing Literature Volume52, Issue12December 2013Pages 1596-1598 RelatedInformation

Bowel-associated dermatosis-arthritis syndrome – a relapsing, extremely rare extraintestinal sign of acute flare-up in Crohn's disease

ejd.2011.1346 Auteur(s) : Agnes Kinyo1 kinyoagnes@gmail.com, Klaudia Farkas2, Tamas Molnar2, Istvan Nemeth1, Erika Varga1, Lajos Kemeny1, Rolland Gyulai1 1 Department of Dermatology and Allergology,University of Szeged, Koranyi fasor 6. H-6720, Szeged, Hungary 2 1st Department of Internal Medicine, University of Szeged, Koranyi fasor 6. H-6720, Szeged, Hungary The incidence of skin manifestations is estimated to be 7.6 % in Crohn's disease (CD) [1, 5]. We report a patient with bowel-associated [...]