{'fraction_non_alphanumeric': 0.022297215861154636, 'fraction_numerical': 0.00771363143304809, 'mean_word_length': 5.142116950407106, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
Henoch-Schonlein purpura in child: a case report and review of the literature

It usually follows an upper respiratory infection caused by group A beta-hemolytic streptococcus, Staphylococcus Aureus, or Mycoplasma. Skin biopsies demonstrate leukocytoclastic vasculitis involving dermal capillaries and venules. It often involves skin and gastrointestinal system and may also cause arthralgia/arthritis. It is characterized by palpable purpura predominantly in gravity dependent areas such as lower limbs and extensor aspects of upper extremities or on pressure points such as buttocks. Gastrointestinal (GI) manisfestations may include abdominal pain, vomiting and diarrhea. Renal involvement may be seen in 30% of cases in the form of hematuria, proteinuria and hypertension. The disease is usually self-limiting and treatment is mainly supportive. Corticosteriods may be used to treat HSP with significant GI or renal involvement. Prognosis for childhood HSP is excellent with most children experiencing an acute, self-limited course lasting on average 4 weeks.


INTRODUCTION
Henoch-Schonlein purpura (HSP), also referred to as immunoglobulin A (IgA) vasculitis, is a small vessel vasculitis that affects children of 3-10 year old age group. Incidence of HSP is estimated at 14-20 per 100,000 children per year and it affects males more than females with a 1.2-1.8:1 male/female ratio. 1 It usually follows an upper respiratory infection caused by group A beta-hemolytic streptococcus, Staphylococcus Aureus, or Mycoplasma. Skin biopsies demonstrate leukocytoclastic vasculitis involving dermal capillaries and venules. It often involves skin and gastrointestinal system and may also cause arthralgia/arthritis. It is characterized by palpable purpura predominantly in gravity dependent areas such as lower limbs and extensor aspects of upper extremities or on pressure points such as buttocks. Gastrointestinal (GI) manisfestations may include abdominal pain, vomiting and diarrhea. Renal involvement may be seen in 30% of cases in the form of hematuria, proteinuria and hypertension.
The disease is usually self-limiting and treatment is mainly supportive. Corticosteriods may be used to treat HSP with significant GI or renal involvement. Prognosis for childhood HSP is excellent with most children experiencing an acute, self-limited course lasting on average 4 weeks.

CASE REPORT
We report a 6 year old female child presented with complaints of rash over both lower extremities involving the buttocks, joint pain, pain in abdomen and multiple episodes of vomiting for 1 day. Rash started from both lower limbs involving the buttocks and later progressing towards the extensor aspect of both upper extremities and was associated with swelling of face.
Patient also presented with joint pain involving both knees not associated with swelling around the joints. Pain in abdomen was sudden, periumbilical, intermittent and not radiating elsewhere. Child developed 8-10 episodes of vomiting, non-projectile, non-bilious, with 1-2 episodes being blood stained containing streaks of blood.
There was no history of fever or any preceding upper respiratory illness in the child.
On examination, general condition of the patient was fair and vitals were stable. Abdomen was soft and non-tender. There was presence of non-tender, non-blanching purpuric rash over both lower extremities (Figure 1a and b) involving buttocks ( Figure 2) and on the extensor aspect of both upper extremities ( Figure 3).
Lab findings were suggestive of Anemia with Hb of 9.5 gm/dl, normal WBC count and thrombocytosis with Platelet count of 5.6 lakhs. Serum electrolytes were within normal limits. Urine routine was normal and there was no evidence of any hematuria or proteinuria.
Renal function tests were normal and stool routine was suggestive of occult blood in stool.
USG abdomen was suggestive of non-necrotic sub-centric abdominal lymphadenopathy and minimal ascites.
Diagnosis of HSP was made as per the European League against Rheumatism (EuLaR) or Pediatric Rheumatology European Society (PRES) criteria. 2,3 Child was treated with oral prednisolone for 5 days, following which child developed subcutaneous edema ( Figure 4) and dyselectrolytemia but child finally responded to Inj Methylprednisolone@2 mg/kg/day which was later tapered and omitted successfully. Child also received other supportive care in the form of anti-emetics, antacids, analgesics and fluids.
Our case did not have any renal involvement manifesting in the form of hematuria, hypertension or frank nephritis. Child also did not develop any neurological manifestations.
Patient was advised follow up in the OPD with serial urine analysis and BP monitoring.    Later Schönlein recognized the association between purpura and arthritis while a case reported by Henoch also included gastrointestinal symptoms along with the renal involvement. 4 HSP is the most common vasculitis of the children. 50% cases occur before the age of five and males are affected almost twice as common as females. 5 The exact etiology and pathogenesis of HSP is yet to be determined. Seasonal variation depicts high prevalence rate in winter and spring and is unusual in summer months. It is also suggested that various triggers like bacterial and viral infections, vaccinations, drugs and autoimmune mechanisms may result in the formation of an antigen and antibody complex and the deposition of such formed immune complex in the small vessels may activate the alternate compliment pathway leading to neutrophil aggregation which results in inflammation and vasculitis. 6 Among all, the preceding infection of β-haemolytic Streptococcus is the most commonly found pattern.
Positive throat cultures as well as increased titres of antistreptolysin O have been often found in many patients. 7 The evidence of prior infection has not been recorded in the present case.
The patient generally presents with the classic tetrad of rashes, poly-arthralgia, abdominal pain, and renal disease. Hallmark of HSP is the non-blanching rash which clinically appears as a palpable purpura on the lower legs and arms. 5 Musculoskeletal involvement is generally characterized by the pain and swelling of the joints, with a predilection for large joints such as the knees and ankles. GI manifestations include abdominal pain followed by vomiting and intestinal bleeding. Microscopic hematuria and albuminuria are the prominent renal findings. 8 Our case had the symptoms of rashes over both lower legs, pain in abdomen and vomiting and pain in both knees indicating GIT and joint involvement. Significant laboratory finding was occult blood test positive in stool. However, there were no signs of hematuria and albuminuria so no renal involvement. The diagnosis of HSP was made by EuLAR or PRES criteria (Table 1). There is a predominantly spontaneous resolution of all symptoms except that of the renal disease in the majority of the cases. Steroids are more often used for the relief of abdominal pain, joint pain and skin disease. Alternatively, methotrexate and dapsone have been quite effective (steroid sparing agent) for the treatment of chronic abdominal pain and skin involvement. 9 The role of the corticosteroids in preventing the long term outcome of renal complications is controversial. Generally prednisolone is the commonly used steroid for the treatment of HSP. The renal involvement has a high morbidity and mortality, otherwise the disease has an excellent prognosis. A systematic review by Narchi et al stated that even if urinalysis is normal at the presentation, patient should undergo follow up urine testing for at least six months as 97% children will develop abnormal urine findings by that time. 10 Rarely few cases of complicated HSP as intussusceptions, gastrointestinal bleeding and with cardiac involvement have been reported. [11][12][13] CONCLUSION HSP being one of the most common vasculitis of the children and its classic presentation of palpable purpura, arthritis, abdominal involvement and renal features makes the diagnosis quite easier. Early initiation of treatment with steroids will help in symptomatic relief and bring a positive outcome. The renal disease may need long term follow up; otherwise the diseases has favourable prognosis.