Interessamento genitale della porpora di Schönlein-Henoch

Henoch-Schönlein purpura (HSP) is a paediatric vasculitis, affecting several organs. Purpura is typically found on posterior surface of lower limbs and is more often associated with joint, abdominal and kidney involvement. The paper reports a case of HSP with mild cutaneous and abdominal manifestations, with genital involvement (haemorrhagic oedema of the penis that is more rarely described in literature), which was treated with oral steroid therapy that led to an optimal clinical response.

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Posterior reversible encephalopathy syndrome in Henoch-Schonlein purpura induced by oral steroid therapy and hypertension

Journal of Pediatric Neurology ◽

10.3233/jpn-2010-0424 ◽

2015 ◽

Vol 08 (04) ◽

pp. 421-424 ◽

Cited By ~ 1

Author(s):

Maria Hatzistilianou ◽

Maria Stamou ◽

Liana Fidani ◽

Afrodites Charitandi ◽

Fani Athanasiadou ◽

...

Keyword(s):

Steroid Therapy ◽

Posterior Reversible Encephalopathy Syndrome ◽

Oral Steroid ◽

Henoch Schonlein Purpura ◽

Posterior Reversible Encephalopathy ◽

Oral Steroid Therapy ◽

Reversible Encephalopathy ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

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Biopsy-proven Henoch-Schönlein purpura nephritis: a single center experience

Pediatric Nephrology ◽

10.1007/s00467-020-04809-8 ◽

2020 ◽

Author(s):

Eda Didem Kurt-Şükür ◽

Thivya Sekar ◽

Kjell Tullus

Keyword(s):

Angiotensin Receptor Blockers ◽

Laboratory Data ◽

Treatment Modalities ◽

Oxford Classification ◽

Henoch Schonlein Purpura ◽

Nephritic Syndrome ◽

Kidney Involvement ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura ◽

Purpura Nephritis

Abstract Background Knowledge on normal progress and treatment of Henoch-Schönlein purpura nephritis (HSPN) is limited. This study reviews outcome, clinical, pathological, and therapeutic factors affecting the prognosis of HSPN patients. Methods Forty-nine children with biopsy-confirmed HSPN diagnosed between September 2008 and 2018 were included. Demographics, clinical and laboratory data, treatment, and outcome were recorded at the time of biopsy, 3, 6, 12, and 24 months and at last visit. Clinical outcome was graded according to Meadow’s criteria. Results The median age at time of biopsy was 10.1 years (IQR:5.7) and female/male ratio 24/25. At presentation, 40.8% of patients had nonnephrotic proteinuria, 18.4% nephrotic syndrome (NS), 4.1% nephritic syndrome (NephrS), and 36.7% NephrS+NS. There were 11 patients with an estimated glomerular filtration rate below 90 ml/min/1.73 m2. Biopsy specimens were classified according to International Study of Kidney Diseases in Children (ISKDC) and Oxford Classification MEST-C scoring systems. Forty-one patients received angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, 37 patients steroids, and 35 patients other immunosuppressive medications. At last visit, 24 patients had stage 1 chronic kidney disease (CKD), three stage 2 CKD, and two had stage 5 CKD. Neither clinical parameters nor ISKDC biopsy grade or treatment modalities effected the final outcome. The Oxford classification showed significantly increased segmental glomerulosclerosis in patients with unfavorable outcome. Favorable outcome was associated with shorter time from kidney involvement to biopsy and start of treatment. Conclusion A large proportion of patients continued to show signs of CKD at last follow-up while only a small proportion developed stage 5 CKD.

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Acute haemorrhagic oedema of infancy: a condition that is not always benign

BMJ Case Reports ◽

10.1136/bcr-2020-236059 ◽

2020 ◽

Vol 13 (11) ◽

pp. e236059

Author(s):

Ugo Cucinotta ◽

Francesca Mazza ◽

Giovanni Battista Pajno ◽

Romina Gallizzi

Keyword(s):

Clinical Outcome ◽

Steroid Therapy ◽

Rare Condition ◽

Henoch Schonlein Purpura ◽

Benign Condition ◽

Atypical Case ◽

Systemic Involvement ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Acute haemorrhagic oedema of infancy (AHOI) is a rare condition and an unusual diagnosis for the paediatrician, as approximately 300 cases have been reported in literature so far. Although it was considered for years a less serious variant of Henoch-Schönlein purpura, nowadays it is thought to be a different entity, with his own characteristics and clinical outcome. In literature it is described as a benign condition, self-limiting and without any systemic involvement in most of the cases. We present an atypical case of AHOI with a severe presentation and which needed an aggressive and prolonged steroid therapy.

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Treatment of Complicated Henoch-Schönlein Purpura with Mycophenolate Mofetil: A Retrospective Case Series Report

International Journal of Rheumatology ◽

10.1155/2010/254316 ◽

2010 ◽

Vol 2010 ◽

pp. 1-5 ◽

Cited By ~ 11

Author(s):

A. A. Nikibakhsh ◽

H. Mahmoodzadeh ◽

M. Karamyyar ◽

S. Hejazi ◽

M. Noroozi ◽

...

Keyword(s):

Mycophenolate Mofetil ◽

Steroid Therapy ◽

Central Nervous System Involvement ◽

Case Series ◽

Systemic Steroid ◽

Henoch Schonlein Purpura ◽

Retrospective Case ◽

Frequent Relapses ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Background. Henoch-Schönlein purpura (HSP) is the most common childhood vasculitis with an incidence of approximately 10 per 100 000 children. There is some evidence to support steroid therapy in the treatment of severe abdominal pain, severe nephritis, and central nervous system involvement. However, the routine use of corticosteroids is controversial. Frequent relapses, lack of response to steroid, steroid dependency, and steroid side effects may occur in some patients. Mycophenolate mofetil (MMF) gains increasing popularity in the treatment of autoimmune disorders, but hitherto, the available evidence to support the use of MMF in HSP is limited to some case study reports.Case Presentation. We report six children with HSP who failed to respond to systemic steroid therapy, whereas MMF successfully treated the manifestations of the disease.Conclusion. The manifestations of HSP disappeared mainly during the first week of treatment with MMF and all the patients were in a complete remission at the end and after discontinuation of the therapy. In our experience, MMF appeared to be safe and effective for the maintenance of remission in the HSP patients.

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Successful Steroid Therapy of Henoch Schonlein Purpura in Young Adult Woman with Moderate Symptoms: A Case Report

Indian Journal of Forensic Medicine & Toxicology ◽

10.37506/ijfmt.v15i3.15987 ◽

2021 ◽

Keyword(s):

Case Report ◽

Young Adult ◽

Steroid Therapy ◽

Adult Woman ◽

Henoch Schonlein Purpura ◽

Young Adult Woman ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

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Henoch-Schonlein purpura masquerading as vesiculobullous lesions in a child

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20205417 ◽

2020 ◽

Vol 8 (1) ◽

pp. 170

Author(s):

Rajeshwari N. ◽

Prahada J. ◽

Kala K.

Keyword(s):

Leukocytoclastic Vasculitis ◽

Lower Limbs ◽

Small Vessel Vasculitis ◽

Direct Immunofluorescence ◽

Henoch Schonlein Purpura ◽

Diagnostic Challenge ◽

Serum Iga ◽

Ulcerative Lesions ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis occurring commonly in children presenting with palpable non-thrombocytopenic purpura, arthralgia and abdominal pain. Haemorrhagic bullous and ulcerative lesions are rare in HSP in children and can be a diagnostic challenge. We present a case of 6-year-old boy child who presented with bullous purpuric lesions in lower limbs, arthralgia and increased serum IgA. Histopathology of skin lesion revealed leukocytoclastic vasculitis. However, the direct immunofluorescence was negative for IgA deposits.

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HLA-DQ and HLA-DRB1 alleles associated with Henoch-Schönlein purpura nephritis in Finnish pediatric population: a genome-wide association study

Pediatric Nephrology ◽

10.1007/s00467-021-04955-7 ◽

2021 ◽

Cited By ~ 1

Author(s):

Mikael Koskela ◽

Julia Nihtilä ◽

Elisa Ylinen ◽

Kaija-Leena Kolho ◽

Matti Nuutinen ◽

...

Keyword(s):

Association Study ◽

Genome Wide Association Study ◽

Reference Population ◽

Genome Wide Association ◽

Henoch Schonlein Purpura ◽

Genome Wide ◽

A Genome ◽

Kidney Involvement ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Abstract Background The pathophysiology of Henoch-Schönlein purpura (HSP) is still unclear, but several findings suggest that genetic factors may influence disease susceptibility. We aimed to perform a genome-wide association study (GWAS) in pediatric HSP patients with an emphasis on severe HSP nephritis. Methods The study included 46 HSP patients, 42 of whom had undergone kidney biopsy. Forty-nine pediatric patients with an inflammatory bowel disease (IBD) served as an autoimmune disease control group while Finnish bone marrow and blood donors represented the general reference population (n = 18,757). GWAS was performed for HSP and IBD samples in a case-control manner against the reference population. The analysis also included imputation of human leukocyte antigen (HLA) alleles. Results GWAS analysis in HSP revealed several polymorphisms from the HLA region that surpassed the genome-wide significance level. Three HLA class II alleles were also significantly more frequent in HSP than in the reference population: DQA1*01:01, DQB1*05:01, and DRB1*01:01. Haplotype DQA1*01:01/DQB1*05:01/DRB1*01:01 occurred in 43.5% of HSP patients, whereas its frequency was 8.2% in IBD patients and 15.0% in the reference population. HSP patients with this haplotype showed similar baseline clinical findings and outcome as HSP patients negative for the haplotype. In IBD patients, no polymorphism or HLA allele appeared significant at the genome-wide level. Conclusions Our results suggest that haplotype DQA1*01:01/DQB1*05:01/DRB1*01:01 is associated with susceptibility to HSP, but not with the severity of the kidney involvement. These HLA associations did not occur in IBD patients, suggesting that they are specific to HSP and not related to susceptibility to autoimmune diseases in general. Graphical abstract

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Clinical significance of anti-phospholipid antibodies in Henoch Schönlein purpura

International Journal of Community Medicine and Public Health ◽

10.18203/2394-6040.ijcmph20213041 ◽

2021 ◽

Vol 8 (8) ◽

pp. 4037

Author(s):

Anuj Gupta ◽

Joshika Agarwal ◽

Shilpi Gupta ◽

Anurag Singh

Keyword(s):

Antibody Titer ◽

Close Association ◽

P Value ◽

Iga Vasculitis ◽

Henoch Schonlein Purpura ◽

Vascular Thrombosis ◽

Kidney Involvement ◽

Intravenous Steroids ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Background: Henoch Schönlein purpura also known as IgA vasculitis is described histologically by IgA deposition in the blood vessel walls and presents with kidney involvement, palpable purpura, arthralgia, and abdominal pain. Our study aims to evaluate the association between anti-phospholipid antibody, anti-cardiolipin antibody, anti-beta(2) glycoprotein 1 antibody and Anti-phosphatidylserine/prothrombin antibodies and IgA vasculitis. Treatment response with intravenous steroids and cyclophosphamide was also studied based on resolution of antibody titer.Methods: We conducted an observational study in three Rheumatology clinics at Ahmedabad, India. Data was collected for a period of 6 months. Diagnosis of IgA vasculitis was determined based on the International Chapel hill consensus conference 2012. Disease activity was assessed based on antibody titer, histological grading and through a pre-determined clinical form to assess objective clinical symptoms. P value of less than 0.05 was considered significantResults: Study evaluated antibody titer of 178 patients. Sixty one percent of the patient's had positive anti-phospholipid antibody titer with predominant antibody subtype as IgG. Inflammatory markers were significantly higher in patient having anti-phospholipid antibody titer. Anti-phospholipid antibody was present in 100 percent patients who had vascular thrombosis. IgG subtype of anti-cardiolipin antibody were found in 60 percent of the patients with renal complication.Conclusions: Anti-phospholipid antibody have a close association with IgA vasculitis. Anti-phospholipid antibody has a significant role in mounting inflammatory response and vascular thrombosis. Combination treatment of intravenous steroids and cyclophosphamide found to be more effective in resolution of titer

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Henoch-Schonlein purpura associated with HLA-B27 positive axial spondyloarthritis in a young man

BMJ Case Reports ◽

10.1136/bcr-2018-228881 ◽

2019 ◽

Vol 12 (5) ◽

pp. e228881 ◽

Cited By ~ 1

Author(s):

Kevin John John ◽

Mohammad Sadiq ◽

Meera Thomas ◽

Vijay Prakash Turaka

Keyword(s):

Axial Spondyloarthritis ◽

Case Reports ◽

Lower Limbs ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Hla B27 ◽

Henoch Schonlein Purpura ◽

Seronegative Spondyloarthropathies ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Axial spondyloarthropathies are characterised by bilateral sacroiliitis, asymmetric oligoarthritis, association with the human leucocyte antigen (HLA)-B27, enthesitis and dactylitis. Although IgA nephropathy has a well-documented association with seronegative spondyloarthropathies, the association with Henoch-Schonlein purpura (HSP) has been documented only in few case reports. The present case is that of a 26-year-old man who presented with fever, lower limb arthritis, abdominal pain, palpable purpura over the buttocks and lower limbs, and clinical features of sacroiliitis. His blood tests showed elevated inflammatory markers and rheumatoid factor was negative. CT scan of the sacroiliac joints confirmed sacroiliitis. Skin biopsy revealed neutrophilic small vessel vasculitis. HLA-B27 was positive in blood. A diagnosis of HSP with HLA-B27 positive axial spondyloarthritis was made. HSP can be associated with HLA-B27 positive axial spondyloarthritis and has to be considered while evaluating for causes of cutaneous small vessel vasculitis in such patients.

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