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      Leukocytoclastic Vasculitis in an Adolescent with New-Onset Crohn’s Disease

      case-report

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          Abstract

          Extraintestinal manifestations frequently affect patients with inflammatory bowel disease. They can involve virtually any organ, with the musculoskeletal and integumentary systems being the most common. Leukocytoclastic vasculitis is a rare extraintestinal manifestation of inflammatory bowel disease, especially at disease onset. It has been reported to occur in association with Crohn’s disease and trimethoprim/sulfamethoxazole (TMP-SMX) exposure independently. We report a case of a 14-year-old female who developed leukocytoclastic vasculitis after exposure to TMP-SMX and was ultimately diagnosed with Crohn’s disease. The patient presented with purpura, oral ulcers, abdominal pain, and intermittent bloody stools. Colonoscopy showed colonic inflammation, and biopsies revealed severe chronic active colitis with crypt abscesses. A skin biopsy confirmed the diagnosis of leukocytoclastic vasculitis. Management consisted of high-dose steroids and infliximab, with resolutions of her symptoms. This case emphasizes that extraintestinal manifestations are multifactorial in nature, with the example of an existing genetic predisposition through Crohn’s disease and a triggering factor such as TMP-SMX.

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          The histological assessment of cutaneous vasculitis.

          Vasculitis is defined as inflammation directed at vessels, which compromises or destroys the vessel wall leading to haemorrhagic and/or ischaemic events. Skin biopsy is the gold standard for the diagnosis of cutaneous vasculitis, whose manifestations include urticaria, infiltrative erythema, petechiae, purpura, purpuric papules, haemorrhagic vesicles and bullae, nodules, livedo racemosa, deep (punched out) ulcers and digital gangrene. These varied morphologies are a direct reflection of size of the vessels and extent of the vascular bed affected, ranging from a vasculitis affecting few superficial, small vessels in petechial eruptions to extensive pan-dermal small vessel vasculitis in haemorrhagic bullae to muscular vessel vasculitis in lower extremity nodules with livedo racemosa. Skin biopsy, extending to subcutis and taken from the earliest, most symptomatic, reddish or purpuric lesion is crucial for obtaining a high-yielding diagnostic sample. Based on histology, vasculitis can be classified on the size of vessels affected and the dominant immune cell mediating the inflammation (e.g. neutrophilic, granulomatous, lymphocytic, or eosinophilic). Disruption of small vessels by inflammatory cells, deposition of fibrin within the lumen and/or vessel wall coupled with nuclear debris allows for the confident recognition of small vessel, mostly neutrophilic vasculitis (also known as leukocytoclastic vasculitis). In contrast, muscular vessel vasculitis can be identified solely by infiltration of its wall by inflammatory cells. Extravasation of red blood cells (purpura) and necrosis are supportive, but not diagnostic of vasculitis as they are also seen in haemorrhagic and/or vaso-occlusive disorders (pseudovasculitis). Vasculitic foci associated with extravascular granulomas (palisaded neutrophilic and granulomatous dermatitis), tissue eosinophilia, or tissue neutrophilia signal the risk for, or co-existence of systemic disease. This essential histological information coupled with direct immunofluorescence and anti-neutrophil cytoplasmic data and clinical findings enables more precise and accurate diagnosis of localized and systemic vasculitis syndromes.
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            Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature.

            Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance.
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              Diagnosis and management of leukocytoclastic vasculitis

              Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch–Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.
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                Author and article information

                Journal
                JPGN Rep
                JPGN Rep
                PG9
                JPGN Reports
                Lippincott Williams & Wilkins, Inc. (Philadelphia, PA )
                2691-171X
                November 2023
                28 August 2023
                : 4
                : 4
                : e359
                Affiliations
                From the [* ]PGY-2, Pediatric Residency Program, Nicklaus Children’s Hospital, Miami, Fl
                []Department of Pathology and Laboratory Medicine, Pediatric Pathologist, Nicklaus Children’s Hospital, Miami, Fl
                []Pediatric Gastroenterology, Hepatology, and Nutrition Division, Nicklaus Children’s Hospital, Miami, Fl.
                Author notes
                Correspondence: Aldo Majluta Yeb, MD, Nicklaus Children’s Hospital, 3100 S.W. 62nd Ave Miami, FL 33155. E-mail: Aldo.majluta@ 123456nicklaushealth.org
                Article
                00010
                10.1097/PG9.0000000000000359
                10684162
                38034455
                200dbc80-0297-4270-a8f0-b7642f6448e3
                Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

                History
                : 20 April 2023
                : 15 June 2023
                Categories
                Case Report
                Gastroenterology Inflammatory Bowel Disease
                Custom metadata
                TRUE

                crohn’s disease,extraintestinal manifestations,leukocytoclastic vasculitis

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