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

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.

scholarly journals Neutrophil-to-lymphocyte Ratio: A Biomarker for Predicting Systemic Involvement in Henoch–Schonlein Purpura

2021 ◽  
Vol 0 ◽  
pp. 1-4
Author(s):  
Wang Lei ◽  
Shan Yun-Yun ◽  
Xu Ai-E
Keyword(s):  
Inflammatory Marker ◽  
Renal Involvement ◽  
Neutrophil To Lymphocyte Ratio ◽  
Henoch Schonlein Purpura ◽  
Systemic Involvement ◽  
Lymphocyte Ratio ◽  
Number Of Patients ◽  
Pre Treatment ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Background: Henoch–Schonlein purpura (HSP) is one of the commonest entities included within the category of cutaneous vasculitis (CV). Our work is purposed to explore the predictive value of neutrophil-to-lymphocyte ratio (NLR) for systemic involvement in Henoch– Schonlein purpura patients. This ratio is known as an inflammatory marker, and is used to assess the systemic inflammation associated with various diseases. Our objective is to establish whether it can be applied for the prediction of renal and gastrointestinal (GI) or purely renal involvement in Henoch–Schonlein purpura. Aim: To determine the relationship between neutrophil-to-lymphocyte ratio and systemic involvement in Henoch–Schonlein purpura Methods: This is a retrospective review of the patients who were diagnosed with Henoch–Schonlein purpura in our hospital between 2012 and 2018. Results: A total of 57 patients met our inclusion criteria. Pre-treatment neutrophil-to-lymphocyte ratio was significantly associated with renal and/or GI manifestations of the disease (p<0.001). The optimal cut-off value of this ratio for predicting systemic involvement was 2.48, with a 95% specificity and a 94% sensitivity. In addition, pretreatment ratio was also found to be significantly correlated with the severity of relevant systemic manifestations of Henoch–Schonlein purpura (r=0.831; p<0.01). Limitations: The small number of patients recruited for our research, its retrospective design, and the inclusion of patients attending the same hospital. Conclusion: This study suggests that neutrophil-to-lymphocyte ratio is suitable as a potential indicator for predicting the systemic involvement in Henoch–Schonlein purpura.

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 ◽  
...  
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.

Interessamento genitale della porpora di Schönlein-Henoch

2021 ◽  
Vol 24 (3) ◽  
pp. 73-74
Author(s):  
Nicola Tovaglieri ◽  
Rossella Amariti ◽  
Francesca Loiacono ◽  
Costantino De Giacomo
Keyword(s):  
Clinical Response ◽  
Steroid Therapy ◽  
Lower Limbs ◽  
Oral Steroid ◽  
Posterior Surface ◽  
Henoch Schonlein Purpura ◽  
Kidney Involvement ◽  
Oral Steroid Therapy ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

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.

A rapid response to steroid therapy in acute scrotum: A case of Henoch-Schönlein purpura

2012 ◽  
pp. 71-74
Author(s):  
Ali Kanık ◽  
Engin Köse ◽  
Kayı Eliaçık ◽  
Seda Şirin Köse ◽  
Nil Cefa Arslan ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Rapid Response ◽  
Acute Scrotum ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

P064 Henoch-Schonlein Purpura: experience of a general pediatric center

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
D J Belamri ◽  
I Boustil ◽  
S Zoubir ◽  
Bensoltane H Chériet ◽  
N Bouchair
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Renal Involvement ◽  
Winter Period ◽  
Henoch Schonlein Purpura ◽  
Severe Renal Failure ◽  
Benign Condition ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Abstract Background Henoch-Schonlein purpura (HSP) is the most classic dysimmune vasculitis in children, related to an abnormal response of an immature immune system following an external antigen attack. Digestive involvement in HSP conditions the immediate vital prognosis and severe renal failure conditions the long-term functional prognosis. The Objective is to identify the epidemiological, clinical, disease course and therapeutic particularities of patients with HSP with a review of the literature. Material and method The authors reported the results of a retrospective study of cases of patients with HSP hospitalized and treated in the 02 pediatric departments of the wilaya of Annaba in Algeria (Clinique Sainte Thérèse, Annaba hospital university center and EHS El Bouni) over a period of 02 years. Information was obtained from hospital records. Results 24 cases of HSP were identified with a sex ratio of 1.4 (14 boys and 10 girls) and a hospital incidence of 1.66%. The average age was 6.6 years. Half of patients was hospitalized during the autumn-winter period, 67% of whom had an ENT infection before their hospitalizations. 17% of cases have personal history of HSP (the oldest was 5 years ago and the most recent 10 days before hospitalization). 7 patients underwent surgeries prior to hospitalization. Skin lesions were found in all patients. Joint, digestive and renal involvement were recorded in 50%, 80% and 25% respectively. Gastrointestinal bleeding has been observed in one patient. Only 1 patient with renal impairment progressed to chronic renal failure and only 1 patient had confirmed hypertension. A rare association with viral hepatitis A has been noted in one patient. Conclusion HSP remains a benign condition, easy to recognize clinically. Its immediate prognosis depends on the digestive complications and its long-term on nephropathy which may expose to the risk of chronic renal failure.

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