4 years old boy developed rashes on legs since days. It is bright red and not itchy.he complains of knee and ankle joint pain.he has no fever or pallor or lymphadenopathy
Rash are increasing in leg and it is slightly raised
his cbc ,peripheral smear is normal,
What is hsp?
Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood and is characterized by leukocytoclastic vasculitis and immunoglobulin A deposition in the small vessels in the skin, joints, gastrointestinal tract, and kidney.
According to the 2012 International Chapel Hill Consensus Conference nomenclature, HSP is also referred to as IgA vasculitis, based on the presence of vasculitis with predomi-nance of IgA deposits affecting small vessels.
What is the pathology?
Skin biopsies demonstrate leukocytoclastic vasculitis of the dermal capillaries and postcapillary venules. The inflammatory infiltrate includes neutrophils and monocytes. Renal histopathology typically shows endocapillary proliferative glomerulonephritis, ranging from a focal segmental process to extensive crescentic involvement. In all tissues, immunofluorescence identifies IgA deposition in walls of small vessels (, accompanied to a lesser extent by deposition of C3, fibrin, and IgM.
What is the PATHOGENESIS?
T he exact pathogenesis of HSP remains unknown. Given the seasonality of HSP and the frequency of preceding upper respiratory infections, infectious triggers such as group A β-hemolytic streptococcus, Staphylococcus aureus, mycoplasma, and adenovirus have been suspected.
The common finding of deposition of IgA, specifically IgA1, suggests that HSP is a disease mediated by IgA and IgA immune complexes. HSP occasionally clusters in families, suggesting a genetic component. HLA-B34 and HLA-DRB1*01 alleles have been linked to HSP nephritis. .
What are the clinical criteria?
EUROPEAN LEAGUE AGAINST RHEUMATISM/PEDIATRIC RHEUMATOLOGY EUROPEAN SOCIETY CRITERIA†
Palpable purpura (in absence of coagulopathy or thrombocytopenia) and 1 or more of the following criteria must be present: •Abdominalpain(acute,diffuse,colickypain)
•Renalinvolvement(proteinuria>3 g/24 hr), hematuria or red cell casts
What are the clinical features?
T he hallmark of HSP is its rash: palpable purpura starting as pink macules or wheals and developing into petechiae, raised purpura, or larger ecchymoses. Occasionally, bullae and ulcerations develop. The skin lesions are usually symmetric and occur in gravity-dependent areas (lower extremities), extensor aspect of the upper extremities or on pressure points
Musculoskeletal involvement, including arthritis and arthralgias, is common, occurring in up to 75% of children with HSP. The arthritis tends to be self-limited and oligoarticular, with a predilection for large joints such as the knees and ankles, and does not lead to deformities.
Gastrointestinal (GI) manifestations occur in up to 80% of children with HSP and include abdominal pain, vomiting, diarrhea, paralyticileus, and melena. Intussusception, mesenteric ischemia, and intestinal perforation are rare but serious complications. Endoscopic evaluation is usually not needed but may identify vasculitis of the intestinal tract.
Renal involvement occurs in up to 30% of children with HSP, manifesting as microscopic hematuria, proteinuria, hypertension, frank nephritis, nephrotic syndrome, and acute or chronic renal failure.
What are the complications?
Acutely, serious GI involvement, including intussusception and intestinal perforation, imparts significant morbidity and mortality.
Renal disease is the major long-term complication, occurring in 1–2% of children with HSP. Renal disease can develop up to 6 mo after diagnosis but rarely does so if the initial urinalysis findings are normal. Therefore, it is recommended that children with HSP undergo serial monitoring of blood pressure and urinalysis for at least 6 mo after diagnosis to monitor for development of nephritis.
What are the d/d?
T he differential diagnosis for HSP depends on specific organ involvement but usually includes other small vessel vasculitides, infections, acute poststreptococcal glomerulonephritis, hemolytic-uremic syndrome, coagulopathies, and other acute intraabdominal processes.
Additional disorders in the differential include papular-purpuric glove and sock syndrome, systemic lupus erythematosus (SLE), other vasculitides (urticarial, hypersensitivity), and thrombocytopenia
What is the treatment?
Treatment for mild and self-limited HSP is supportive, with an emphasis on ensuring adequate hydration, nutrition, and analgesia.
Corticosteroids are most often used to treat significant GI involvement or other lifethreatening manifestations. Glucocorticoids such as oral prednisone (1-2 mg/kg/day), or in severe cases, intravenous (IV) methylprednisolone for 1-2 wk, followed by taper, reduce abdominal and joint pain but do not alter overall prognosis.
Corticosteroids are not routinely recommended for prevention of complications such as nephritis. Rapid tapering of corticosteroids may lead to a flare of HSP symptoms. Although few data are available to demonstrate efficacy, intravenous immune globulin (IVIG) and plasma exchange are sometimes used for severe disease.
In some patients, chronic HSP renal disease is managed with a variety of immunosuppressants, including azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. ESRD develops in <5% of children with HSP nephritis.
Please talk to the mother who has these queries
Will my child be alright?
Will it recurr?
Will it cause side effects?
Will it affect kidney?
Overall, the prognosis for childhood HSP is excellent, and most children experience an acute, self-limited course lasting on average 4 wk.
However, 15–60% of children with HSP experience 1 or more recurrences, typically within 4-6 mo of diagnosis.
With each relapse, symptoms are usually milder than at presentation. Children with a more severe initial course are at higher risk for relapse.
The long-term prognosis usually depends on the severity and duration of GI or renal involvement.
Chronic renal disease develops in 1–2% of children with HSP, and <5% of those with HSP nephritis go on to have ESRD.
POST TEST QUESTIONS
one more options could be correct
Which all are features of classic HSP
deformity forming arthritis
Chronic kidney disease
Which all infection are implicated as triggers?
Lab features of HSP include
Serum is A elevated
Urine protein +
Indication for steroid
Severe gi bleed
None of the above
What is leucocytoclasis?
Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis and hypersensitivity angiitis, is a histopathologic term commonly used to denote a small-vessel vasculitis (see the image below).  Histologically, LCV is characterized by leukocytoclasis, which refers to vascular damage caused by nuclear debris from infiltrating neutrophils. LCV classically presents as palpable purpura. Less common clinical findings include urticarial plaques, vesicles, bullae, and pustules