A case of purpuric skin rash

DR SAIMA BATOOL NAQVI

ASSISTANT PROFESSOR PEDIATRICS
• Name:Mahnoor
• Age:4 yrs
• address:raiwind lhr.
• Presenting complaints:
a reddish purple rash on legs…..1 day
History of present illness:
A reddish purple rash on legs with itching.some of
the lesions were ulcerated due to excessive itching
by the child.
Similar episode of rash about two weeks after initial
presentation .
• Swelling of both legs .
• Pain in knee joints.
• Complaint of diffuse abdominal pain.
Past history:Insignificant .
Birth history:NVD at hospital with normal birth
events.
Development history:normal
Vaccination :complete course according to EPI.
 Examination
A well looking average built girl with reddish
purple ,purpuric rash on the legs and buttocks.
Vitals: stable. BP: Normal.
Weight:12 kg.
Height:105 cm.
H.C:50 cm.
• Systemic examination:
normal.
Abdominal examination:
no tenderness,no mass,no vicseromegaly.
 Laboratory investigations
• CBC:
WBC :12,000/hpf. P:78%,L:20%
HB:13.6 G/dl.
PLT:407,000/hpf.
ESR:45mm .
• P.SMEAR:
• MCV :80 µ m³ normal.
MCH:26pg/cell.
MCHC:34g/dl.
• LFTs:s.bilirubin:0.4mg/dl.
ALP:223 IU.
ALT:30 IU.
AST:32 IU.
• ASOT:normal.
• S.c3 level:100 mg/dl(55-120)
• Abd.usg:normal
• RFTs:s.urea:35mg/dl.
s.creatinin:0.6mg/dl.
• URINE C/E:NORMAL.
• ANA:negative.
• RF:negative.
• CXR:normal.
 Treatment
• Oral acetaminophen.
• Avoidence of trauma.
• Oral steroids:1mg/kg /day in three divided
doses.tapering gradually over a period of 6-8
weeks.
• Follow up:for renal complications………….
which may appear later as well.urine
C/E…,monthly for 1 yr.
 HENOCH SCHONLEIN PURPURA
(HSP)
• ANAPHYLACTOID PURPURA.
• Common vasculitis of small vessels.
• Non thrombocytopenic purpura.
• SCHONLEIN was a German physician.in 1837 he
described a disease with purpura and rheumatic
involvement and named it “purpura
rheumatica”.HENOCH was his student.
• Speculated that famous musician MOZART died
of HSP.
 Epidemiology
• 14-15/100,000 CHILDREN /yr.
• Age:2-8 yrs.
• Winter ,spring .
• Male:Female ratio: 1:0.7.
 Pathogenesis
• Multofactorial …genetic,
environmental,antigenic causes.
• HLA DRB1*01 association.
• INCREASE amount of cytokines.TNF-alpha,IL-6.
• INCREASE ASOT.
• IgA mediated vasculitis of small vessels of the
skin,renal glomeruli,GIT,JOINTS.
• ALLERGENS:
• food,horse serum,drugs etc.
• Infections: streptococcus gp A,
Hib,salmonella,shigella,parainfluenza,
mycoplasma,EBV,varicella,measles etc.
• drugs:ampicillin,erythrocin,penicillin,quinine etc.
 Clinical features
• Acute onset.
• Low grade fever.
• Typical rash:initially….pinkish
maculopapules(blanch on pressure) urticarial.
• Petechiae or purpura.
• Colour changes:red to purple to rusty brown.
• last for 3-10 days.
• Appear at few days to 3-4 months interval.
• 50% risk of recurrence within 6 weeks of
interval.
• Dependent oedema.nonpitting.
 Arthritis
• In 2/3 cases.
• Knees and ankles.
• Serous ,nonhemorrhagic effusions.
• Nondeforming.
 GIT
• intermittent colicky abdominal pain.
• Occult heme +ve stools or bloody diarrohea.
• Hemetemesis.
• INTUSSUSCEPTION. It is due to submucosal
hematomas and are usually enteroenteral so
difficult to reduce by enema and requires
surgical reduction and or resection.
 RENAL INVOLVEMENT
• In 20-25% cases.
• hematuria,proteinuria,nephritis or
nephrosis,ARF.
• Chronic hypertension or ESRD.
 NEUROLOGICAL
• RARE.
• Seizures,paresis,coma.
 Rare complications
• Rheumatoid –like nodules.
• Cardiac and eye involvement.
• Mononeuropathies.
• Pancreatitis.
• pulmonary or I/M hemorrhage.
• Skin rash……95-100%.
• GIT…………….70-75%.
• JOINTS……….45-50%.
• KIDNEYS…….25-30%.
 Differential diagnosis
• Kawasaki disease.
• Systemic onset JRA.
• FMF…familial mediterranean fever.
• Inflammatory bowel disease….crohn
• Meningococcemia.
• SLE
• Acute hemorrhagic oedema.(AHE)
• Poly arteritis nodosa.(PAN)
 Laboratory investigations
Clinical diagnosis.
• CBC.ESR,CRP.
• IgM and IgA : elevated.
• ANA and RF……NEGATIVE.
• PT,APTT:normal.
• Anticardiolipin ,antiphospholipid antibody.
• Abdominal x-rays plain.
• Abdominal USG.
• Barium enema.
• Urine c/e.
• Skin biopsy:(if clinical picture is atypical )
leucocytoclastic vasculitis.
• RENAL biopsy:mesengial deposits of IgA
,IgM.C3 and fibrin.
• MRI BRAIN:
 TREATMENT
• Symptomatic :
• Adequate hydration.
• pain control with acetaminophen.
• Avoid competitive sports,avoid lower
extremities in dependent position.
• Scrotal oedema:elevation.
 SPECIFIC TREATMENT
• GIT HEMORRHAGE: corticosteroids:
• Prednisolone :1-2 mg/kg/day oral or i/v for 7-
10 days.
• INTUSSUSCEPTION:surgical reduction,AVOID
hydrostatic reduction due to risk of
perforation.
 Renal involvement
• It will depend upon the presentation of child.
• Rheumatoid nodules:
alternate day colchicine 0.6 mg
on alternate days.
IF positive anticardiolipin and antiphospholipid
antibodies are there then aspirin has to be
given.
 Chronic or recurrent HSP
• Pulses of METHYPREDNISOLONE:
• INDUCTION: I/V methyl
prednisolon30mg/kg/day ……………3-7 days
daily.
• PLUS
• Cyclo phosphamide……1-3mg/kg/day PO.
• MAINTAINANCE:
• Prednisolone… every other day.
• PLUS
• Cyclophosphamide….. .as above.
• TAPERING PREDNISOLONE .
• DISCONTINUE prednisolone over at least 6
months….abruptly stop cyclophosphamide.
 Indications for steroids
1. Persistant nephrotic syndrome.
2. Crescents in more than 50% of glomeruli.
3. Severe abd. Pain.
4. Substantial GI hemorrhage.
5. Severe soft tissue oedema.
6. Neurologic symptoms.
7. Intrapulmonary hemorrhage.
8. Coronary artery vasculitis.
 Complications
• Persistent hypertension.
• ESRD.
• Intussusception.(1-5%).
• Protein losing enteropathy.
• Hemorrhagic pancreatitis.
• Hydrops of gall bladder.
• Stricture of oesophagus.
• Ileus.
• Bowel perforation,ischemia, infarction.
• Pseudomembranous colitis.
• Appendicitis.
• Skin necrosis.
• Subarachnoid,subdural,and cortical
hemorrhage and infarction.
• Peripheral mono or polyneuropathies(GBS).
• pulmonary hemorrhage.
• Testicular torsion.
 Follow up
• If normal renal functions and urinalysis:6
monthly follow up with urine examination.
• If nephotic or nephritic picture on urinalysis:
Urine c/e…..weekly for 1 month.
than ……….alternate weekly for 2nd month.
than………..monthly for 1 year.
 Prognosis
• A self limited vasculitis excellent overall
prognosis.
• IF RENAL involvement….<1 % have persistant
renal disease.
• <0.1% develop serious renal disease or ESRD.
• RARELY death due to bowel infarction,CNS
involvement or ESRD.
• LOOK for features of other C.T diseases .
THANK YOU