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

2019 ◽  
Vol 12 (5) ◽  
pp. e228881 ◽  
Author(s):  
Kevin John John ◽  
Mohammad Sadiq ◽  
Meera Thomas ◽  
Vijay Prakash Turaka

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.

2020 ◽  
Vol 8 (1) ◽  
pp. 170
Author(s):  
Rajeshwari N. ◽  
Prahada J. ◽  
Kala K.

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. 


2019 ◽  
Vol 7 (11) ◽  
pp. 1812-1814
Author(s):  
Bella Kurnia

BACKGROUND: Henoch- schonlein purpura (HSP) is an IgA- mediated systemic small vessel vasculitis. It is the most common form of systemic vasculitis in children.CASE REPORT: A 9 years old girl admitted to the hospital with chief complain of purplish red rash on both legs since approximately 1 week with painful knees and ankles that make the patient unable to walk. The patient was diagnosed with HSP and was treated with corticosteroid and analgesics. The patients only stayed for 2 nights at the hospital and discharged from the hospital with the ability to walk and experience no pain. CONCLUSION: The role of corticosteroids in the treatment of HSP is still controversial. But from various research, we can conclude that the role of corticosteroid in HSP is as a symptom reliever (reduce abdominal pain and arthritis), but does not slow the progression of renal disease.


Author(s):  
Xiang Shi ◽  
Wen-Chao Li ◽  
Li-Jun Mo ◽  
Xiao-Hong Li ◽  
Yu-Zhen Luo ◽  
...  

Background Henoch-Schonlein purpura is a systemic small-vessel vasculitis that occurs mainly in children. A review of the literature has suggested a correlation between mean platelet volume and several inflammatory disorders. However, to the best of our knowledge, any potential correlation between mean platelet volume and Henoch-Schonlein purpura has not been reported in the literature. Therefore, our study aimed to evaluate the role of mean platelet volume concentrations in patients with Henoch-Schonlein purpura. Methods This study included 97 children with Henoch-Schonlein purpura and 120 healthy individuals as controls. Results Mean platelet volume concentrations were found to be significantly lower in Henoch-Schonlein purpura patients compared with healthy controls (8.1 ± 0.86 vs. 9.4 ± 0.81, P < 0.001). Similarly, significant negative correlations were observed between mean platelet volume and neutrophil count, platelet count and erythrocyte sedimentation rate in patients with Henoch-Schonlein purpura (r=−0.327, P = 0.001; r=−0.419, P < 0.001; r=−0.255, P = 0.012). Interestingly, mean platelet volume was significantly lower in the acute phase compared with the convalescent phase of Henoch-Schonlein purpura patients (7.8 ± 0.86 vs. 8.3 ± 0.77, P = 0.002). A cut-off value for mean platelet volume was 7.85 with area under the curve of 0.726 to identify acute phase vs. convalescent phase in patients with Henoch-Schonlein purpura. Mean platelet volume was independently associated with Henoch-Schonlein purpura in logistic regression analysis (odds ratio = 0.114, 95% confidence interval = 0.053–0.243, P < 0.001). Conclusions Our results suggest that mean platelet volume is inversely associated with disease in patients with Henoch-Schonlein purpura, and mean platelet volume may be a useful marker to identify active disease in Henoch-Schonlein purpura patients.


2012 ◽  
Vol 4 (2) ◽  
pp. 333-335 ◽  
Author(s):  
S K Samanta ◽  
N Mahapatra ◽  
B Aich ◽  
N Sarkar ◽  
A Chatterjee

Introduction: Henoch-Schonlein purpura (HSP) is one of the most common causes of small vessel vasculitis in children, but sometimes may have an atypical presentation. Objective: To report an unusual case of transient cortical blindness in a patient with Henoch-Schonlein purpura. Case: A 3-year-old female child was brought with the complaint of diffuse abdominal pain and hematochezia, which was preceded by high grade fever and cough. Three days later she developed hematuria, hematemesis, melena and hemoptysis along with palpable purpura. Four days later she became irritable and developed a few episodes of generelized tonic clonic seizure, followed by cortical blindness. The CT scan of the brain showed bilateral nonenhancing occipital hypodensity. The magnetic resonance venography showed thrombosis in transverse and sigmoid sinus. She was treated with corticosteroids and her mental status and vision improved. Conclusion: The HSP can cause transient cortical blindness, and recovery is good if therapy is initiated at the appropriate time.DOI: http://dx.doi.org/10.3126/nepjoph.v4i2.6556 Nepal J Ophthalmol 2012; 4 (2): 333-335


2019 ◽  
Vol 7 ◽  
pp. 2050313X1985035
Author(s):  
Farah Kassam ◽  
Sabrina Nurmohamed ◽  
Richard M Haber

Leukocytoclastic vasculitis is the most common form of cutaneous vasculitis. It is a neutrophilic small vessel vasculitis resulting from the deposition of circulating immune complexes. Henoch-Schonlein purpura is a systemic type of leukocytoclastic vasculitis, characterized by immunoglobulin A-mediated blood vessel injury. We present a case of Henoch-Schonlein purpura in an adult female manifesting with a vasculitic rash with Koebner phenomenon.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Visvalingam Arunath ◽  
Arjuna Salinda Athapathu ◽  
Thabitha Jebaseeli Hoole ◽  
Heshan Aruppala ◽  
Asanka Rathnasri ◽  
...  

Henoch–Schönlein purpura is a small vessel vasculitis that usually presents with palpable purpura, arthritis, abdominal pain, and nephritis. Subcutaneous oedema of dependent areas is common; however, oedema in the scalp is extremely rare especially in children older than two years. Here, we report a child with massive disfiguring scalp and facial oedema due to Henoch–Schönlein purpura. An eight-year-old boy presented with characteristic palpable purpuric rash and extensive disfiguring scalp and facial swelling for five days. He complained of blurred vision, vomiting, and severe headache on the day of admission. Examination revealed an ill child with extensive oedema of the face and scalp that was tender on palpation. His blood pressure was above the 99th percentile, and he had exaggerated deep tendon reflexes and extensor plantar responses. All biochemical investigations including renal function tests were normal. Noncontrast CT head showed normal brain, with marked soft tissue swelling of the scalp. Ultrasonography showed soft tissue oedema within and surrounding facial muscles without evidence of neck vessel compression. Urine analysis revealed microscopic haematuria on day 14 of the illness, and immunohistochemical staining of renal biopsy confirmed Henoch–Schönlein purpura nephritis. In conclusion, this case report presents a child with severe, disfiguring scalp and facial oedema due to Henoch–Schönlein purpura. It highlights that severe subcutaneous oedema of Henoch–Schönlein purpura can involve any part of the body not limiting to dependent areas.


2004 ◽  
Vol 31 (8) ◽  
pp. 655-660 ◽  
Author(s):  
Hideki Maejima ◽  
Kyoumi Shirai ◽  
Yumi Shimamura ◽  
Harumi Harada ◽  
Hikaru Eto

Sign in / Sign up

Export Citation Format

Share Document