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      Bullous Henoch-Schönlein Purpura and Associated Nephritis: A Pathological Case Report

      case-report
      1 , , 2 , 3 , 1
      ,
      Cureus
      Cureus
      immunofluorescence, glomerulonephritis, nephropathology, bullous dermatosis, henoch-schönlein purpura

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          Abstract

          Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood, presenting with purpura, predominantly of the lower extremities and occasionally with renal involvement as well. Although associated with childhood, HSP, although rarely, can also develop in adults as well. Here we present a patient in his sixties, presenting with a myriad of rash units on his lower extremities, including bullous ones, and a constellation of chronic kidney failure. Skin and renal biopsy specimens revealed morphological changes and immune depositions representative of HSP. Despite treatment, the patient's kidney failure slowly progressed, and he expired eight months after his presentation due to associated complications. Although rare, the bullous form of HSP can be viewed as a more aggressive form of the disease, as seen by the presentation constellation and rapid progression in our case.

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          Most cited references18

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          Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins.

          The frequency and ethnic variation of Henoch-Schönlein purpura, Kawasaki disease, and rarer vasculitides during childhood are not well characterised. Our aim was to ascertain the incidence and ethnic distribution of these conditions in children resident in a region of the UK with a diverse ethnic mix. 1.1 million children younger than age 17 years live in the West Midlands. Between Sept 1, 1996, and Aug 31, 1999, we surveyed this population with monthly questionnaires sent to 321 consultants, a single questionnaire sent to 2860 family doctors, and review of 406 case notes with diagnostic codes for vasculitis. We included in the analyses children who fulfilled established criteria for vasculitis with disease onset during the study, and calculated incidence rates from population rates derived from the census of 1991. We identified 586 new instances of vasculitis and connective tissue disease. The estimated annual incidence of Henoch-Schönlein purpura was 20.4 per 100000, and was highest between the ages of 4 years and 6 years (70.3 per 100000). The estimated annual incidence of Kawasaki disease was 5.5 per 100000 in children younger than 5 years, and was highest in Indian subcontinent Asian children (14.6 per 100000). Indian subcontinent Asian and black children had the highest incidence of systemic lupus erythematosus, juvenile dermatomyositis, and other primary systemic vasculitides. Childhood Henoch-Schönlein purpura is more frequent in the West Midlands than previously reported, and Kawasaki disease has a higher incidence than previously indicated in the UK, with the highest incidence in Indian subcontinental Asian children. Other vasculitis is rare in childhood.
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            Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review.

            H Narchi (2005)
            The duration of follow up to assess the risk of long term renal impairment in Henoch-Schönlein purpura (HSP) without nephritic or nephrotic syndrome or renal failure on diagnosis remains undetermined. To undertake a systematic review of the literature to assess whether the risk of long term renal impairment without renal involvement on diagnosis could be estimated and to determine the time period when renal involvement is very unlikely after the diagnosis of HSP. Search of studies of unselected children with HSP, and available information on urinary findings, renal involvement, and long term renal function follow up. Studies of selected children with HSP nephropathy at diagnosis were excluded. Twelve studies of 1133 children were reviewed. The follow up period ranged from 6 weeks to 36 years. Proteinuria and/or haematuria, which occurred in 34.2%, of which only one fifth were in association with nephritic or nephrotic syndrome, developed in 85% of cases within 4 weeks of the diagnosis of HSP, in 91% within 6 weeks, and in 97% within 6 months. Permanent renal impairment never developed after normal urinalysis; it occurred in 1.6% of those with isolated urinary abnormalities, and in 19.5% of those who developed nephritic or nephrotic syndrome. No long term renal impairment occurred after normal urinalysis. Even if urinalysis is normal at presentation, the testing should be continued for six months. There is no need to follow up after the first six months those whose urinalysis remains normal.
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              Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial.

              To evaluate the efficacy of early prednisone therapy in preventing renal and treating extrarenal and renal symptoms in Henoch-Schönlein purpura (HSP) in a placebo-controlled trial. A total of 171 patients (84 treated with prednisone and 87 receiving placebo) were included and followed up for 6 months. The endpoints were renal involvement at 1, 3, and 6 months and healing of extrarenal symptoms. The analyses were performed on an intent-to-treat basis. Prednisone (1 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) was effective in reducing the intensity of abdominal pain (pain score, 2.5 vs 4.8; P = .029) and joint pain (4.6 vs 7.3; P = .030). Prednisone did not prevent the development of renal symptoms but was effective in treating them; renal symptoms resolved in 61% of the prednisone patients after treatment, compared with 34% of the placebo patients (difference = 27%; 95% confidence interval = 3% to 47%; P = .024). The general use of prednisone in HSP is not supported, but patients with disturbing symptoms may benefit from early treatment, because prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                16 February 2023
                February 2023
                : 15
                : 2
                : e35051
                Affiliations
                [1 ] General and Clinical Pathology, Forensic Medicine and Deontology, Medical University of Varna, Varna, BGR
                [2 ] Nephrology, St. Marina University Hospital, Varna, BGR
                [3 ] General and Clinical Pathology, St. Marina University Hospital, Varna, BGR
                Author notes
                Article
                10.7759/cureus.35051
                10024341
                36942172
                f4f96881-7536-41a9-a704-76c86bba5735
                Copyright © 2023, Popov et al.

                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
                : 16 February 2023
                Categories
                Pathology
                Allergy/Immunology
                Nephrology

                immunofluorescence,glomerulonephritis,nephropathology,bullous dermatosis,henoch-schönlein purpura

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