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What is Henoch Schoenlein Purpura is characterized by following except: and explain in detail?
Ans D. Thrombocytopenia. (Ref. H - 17th / pg. 2128)# Purpura in 'Henoch Scholein purpura' is due to vasculitis and not thrombocytopenia.HENOCH-SCHONLEIN PURPURA (anaphylactoid purpura)Definition:# It is a small vessel vasculitis, is a distinct systemic vasculitis syndrome characterized by:- palpable purpura, arthralgias, GI involvement and glomerulonephritis.Incidence and Prevalence# It is usually seen in children; most patients range in age from 4-7 years; however, the disease may also be seen in infants and adults.# It is not a rare disease.# The male-to-female ratio is 1.5:1.# A seasonal variation with a peak incidence in spring has been noted.Pathology and Pathogenesis# A immune-complex deposition disease.# A number of inciting antigens have been suggested including upper respiratory tract infections, various drugs, foods, insect bites, and im- munizations. IgA is the antibody class most often seen in the immune complexes and has been demonstrated in the renal biopsies of these patients.Clinical and Laboratory Manifestations# Palpable purpura (most commonly distributed over the buttocks and lower extremities and in pediatric patients, palpable purpura is seen in virtually all patients),# Arthralgias (most patients develop poly- arthralgias in the absence of frank arthritis),# GI signs and symptoms and# Glomerulonephritis (occurs in 10-50% of patients).# Myocardial involvement can occur in adults but is rare in children.# Lab studies show a mild leukocytosis, a normal platelet count, and occasionally eosinophilia. Serum complement components are normal, and IgA levels are elevated.Diagnosis# The diagnosis of Henoch-Schonlein purpura is based on clinical signs and symptoms.# Skin biopsy useful in confirming leukocytoclastic vasculitis with IgA and C3 deposition.# Renal biopsy is rarely needed for diagnosis but may provide prognostic information in some.Prognosis# The prognosis of Henoch-Schonlein purpura is excellent.# Mortality is exceedingly rare, and 1-5% of children progress to end-stage renal disease.# Most patients recover completely, and some do not require therapy.Rx:# Rx is similar for adults and children. When glucocorticoid therapy is required, prednisone, in doses of 1 mg/kg per day and tapered according to clinical response; however, it has not proven beneficial in the Rx of skin or renal disease and does not appear to shorten the duration of active disease or lessen the chance of recurrence.# Patients with rapidly progressive glomerulonephritis have been anecdotally reported to benefit from intensive plasma exchange combined with cytotoxic drugs.