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8/2/2019 Henoch-Schonlein Purpura Discussion
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HENOCH-SCHONLEIN PURPURA
named after Eduard Heinrich Henoch(18201910), a German pediatrician,and his teacher.. Johann Lukas
Schnlein (17931864), who describedit in the 1860s
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A.K. A.
Anaphylactoid Purpura
Purpura rheumatica
systemic small-vessel vasculitis characterizedby deposition of immune complexes containing the
antibody IgA
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Classification
2006 European League Against Rheumatism(EULAR) and Pediatric Rheumatology Society(PReS) classification:
palpable purpura, together with at least 1 of the ff:
diffuse abdominal pain
predominant IgA deposition (biopsy)
acute arthritis in any joint
renal involvment (hematuria, proteinuria)
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Presentation
Classic of HSP:
purpura (hallmark)
arthritis (80%)abdominal pain (62%)
low grade fever and fatigue (more than ofaffected children)
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Rash
pinkish maculopapules that initiallyblanch on pressure
petechia/purpura
(characterized as palpable purpura that evolve
from red to purple to rusty brown before theyeventually fade)
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Rash
Purpura typically appear on the legs andbuttocks, but may also be seen on the arms,face, and trunk
Lesions tend to occur in crops, last from 3-10days
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Arthritis
Usually localized to the knees and ankles
Non-erosive and hence causes no permanentdeformity
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Abdominal pain
Colicky in character, and may beaccompanied by nausea, vomiting,constipation, or diarrhea
There may be blood or mucus in the stools
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Other manifestations
25-50% of cases have evidence of kidneyinvolvement mainly in the form of hematuria
Rare CNS involvement
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Pathophysiology
Complexes of IgA and C3 are deposited onarterioles, capillaries, and venules
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Pathophysiology
PREFERENTIAL
INVOLVEMENT
SKIN JOINTS GIT
PURPURA JOINT PAIN ABDOMINAL PAIN
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HSP can develop after infections withstreptococci (-haemolytic, Lancefield groupA), hepatitis B, herpes simplex virus,parvovirus B19, Coxsackievirus, adenovirus,Helicobacter pylori,measles, mumps, rubella,mycoplasma and numerous others
http://en.wikipedia.org/wiki/Streptococcushttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Hepatitis_Bhttp://en.wikipedia.org/wiki/Herpes_simplex_virushttp://en.wikipedia.org/wiki/Parvovirus_B19http://en.wikipedia.org/wiki/Coxsackievirushttp://en.wikipedia.org/wiki/Adenovirushttp://en.wikipedia.org/wiki/Helicobacter_pylorihttp://en.wikipedia.org/wiki/Measleshttp://en.wikipedia.org/wiki/Mumpshttp://en.wikipedia.org/wiki/Rubellahttp://en.wikipedia.org/wiki/Mycoplasmahttp://en.wikipedia.org/wiki/Mycoplasmahttp://en.wikipedia.org/wiki/Rubellahttp://en.wikipedia.org/wiki/Mumpshttp://en.wikipedia.org/wiki/Measleshttp://en.wikipedia.org/wiki/Helicobacter_pylorihttp://en.wikipedia.org/wiki/Adenovirushttp://en.wikipedia.org/wiki/Coxsackievirushttp://en.wikipedia.org/wiki/Parvovirus_B19http://en.wikipedia.org/wiki/Herpes_simplex_virushttp://en.wikipedia.org/wiki/Hepatitis_Bhttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus_pyogeneshttp://en.wikipedia.org/wiki/Streptococcus8/2/2019 Henoch-Schonlein Purpura Discussion
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Diagnosis
Lab tests neither specific nor diagnostic Blood tests may show:
elevated creatinine and urea levels (in kidneyinvolvement)
raised IgA levels (in about 50%)
and raised C-reactive protein (CRP) orerythrocyte sedimentation rate (ESR) results
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Platelet count may be raised, anddistinguishes it from diseases where lowplatelets are the cause of the purpura
Renal involvement is manifested by RBC,WBC, casts, or albumin in the urine
Diagnosis
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DEFINITIVE DIAGNOSIS of vasculitis:
confirmed by biopsy
Diagnosis
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Management
Symptomatic treatment
adequate hydration, pain control withacetaminophen
Therapy with oral or IV corticosteroids (1-2mg/kg/day)
Alternate-day colchicine (0.6 mg/24hr everyother day) for rheumatoid nodules
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Complications
nephrotic syndrome
bowel perforation
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Prognosis
HSP is a self-limited vasculitic disease with anexcellent overall prognosis (spontaneousrecovery in 94% of children )
In children under 10, the condition recurs inabout 1/3 of cases and usually within 1st
4months after the initial attack
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HSP is systemic small vessel vasculitis
characterized by deposition of immunecomplexes containing the antibody IgA
It occurs mainly in young children and teens
Typical symptoms include palpable purpura,joint pains, and abdominal pain
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Most cases are self-limiting, but the disease
may relapse in a third of cases
The exact cause of HenochSchnlein
purpura is unknown, although it may occurafter certain viral and bacterial infections, aswell as an adverse drug reaction to somemedications
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THANK YOU!
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