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

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
A. A. Nikibakhsh ◽  
H. Mahmoodzadeh ◽  
M. Karamyyar ◽  
S. Hejazi ◽  
M. Noroozi ◽  
...  

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.

2020 ◽  
Vol 13 (11) ◽  
pp. e236059
Author(s):  
Ugo Cucinotta ◽  
Francesca Mazza ◽  
Giovanni Battista Pajno ◽  
Romina Gallizzi

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.


2015 ◽  
Vol 54 (9) ◽  
pp. 900-903 ◽  
Author(s):  
Theodore Chou ◽  
Valerie R. Louissant ◽  
Alexa Adams ◽  
Sevgi Gurkan ◽  
Dalya Chefitz ◽  
...  

2020 ◽  
Author(s):  
Maria Francesca Gicchino ◽  
Dario Iafusco ◽  
Maria Maddalena Marrapodi ◽  
Rosa Melone ◽  
Giovanna Cuomo ◽  
...  

Abstract Background : Henoch Schonlein purpura (HSP) is an acute small vessel vasculitis. It is the most common vasculitis in children. Although the cause is unknown, IgA seems to play a central role in the pathogenesis of Henoch Schonlein purpura. The major clinical features include a palpable purpuric rash on the lower extremities, abdominal pain or renal involvement, and arthritis. Cutaneous manifestations are the essential elements in the diagnosis of Henoch Schonlein purpura. The palpable purpura is characteristically 2 to 10mm in diameter and is usually present on the lower extremities. There are no specific diagnostic tests available for diagnosing this condition. Laboratory studies are useful to exclude other conditions that may mimic Henoch Schonlein purpura. In majority of the cases, the disease is self-limited. Relapsing can occur, in particular during the first year of the disease. There is no consensus on a specific treatment. Corticosteroids are effective in rapid resolution of renal and abdominal manifestations. Immunosuppressive drugs, such as Mycophenolate Mofetil may be a better treatment choice in case of renal involvement.Case report : We report a case of a 14 years old girl affected from recurrent Henoch Schonlein Purpura. From the age of nine years patient presented three episodes of purpura with gastrointestinal involvement, in particular hematemesis, abdominal pain and diarrhoea. Each episode was treated with high doses of corticosteroids (methylprednisolone in vein or prednisone per os). Patient came to our Department during the third episode of Purpura. In consideration of the recurrence of the Henoch Schonlein Purpura and the gastrointestinal involvement we decided to start Mycophenolate Mofetil treatment. Patient’s conditions improved thanks to Mycophenolate Mofetil treatment. Conclusion: In our case of recurrent HSP Mycophenolate Mofetil treatment has been very effective, avoiding the adverse events of a prolonged steroid treatment. This experience teaches us that immunosuppressive agents may be very useful to induce and maintain remission not only in renal involvement, but in all cases of persistence, recurrence or complicated forms of Henoch Schonlein purpura in children.


2012 ◽  
Vol 59 (5) ◽  
pp. 663-669 ◽  
Author(s):  
Jean-François Augusto ◽  
Johnny Sayegh ◽  
Laurence Delapierre ◽  
Anne Croue ◽  
Fréderic Tollis ◽  
...  

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