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PROF.DR.

NOORUL AMEEN
DR.R.PANDICHELVAN
M6

23 yrs old male patient admitted on 5.2.11


in surgical ward with
H/o abdominal pain 14 days
more in right lower quadrant
colicky
intermittent pain
no radiation
not related with food intake

No h/o vomiting
No h/o fever
No h/o diarrhoea
No h/o jaundice
No h/o cough
No h/o dysuria

On examination pt Conscious
Oriented
Afebrile
Ao /Jo/Cy o/Cl

CVS S1S2
RS NVBS
fossa

P/A tenderness in right iliac


CNS NFND

clinically diagnosed as
Acute appendicitis and treated with
NPO
IVF DNS and NS
INJ Ciprofloxin 200mg iv bd
INJ Metronidazole 500mg iv tds
INJ Ranitidine 50 mg iv bd
Planned for surgery

Patient developed skin

rashes in both lower


extremities and physician
opinion was obtained for a/c
appendicitis with ? drug
allergy and fitness for surgery

General examination:
Pt
Conscious
Oriented
Afebrile
I * P * CY * CL * LNE *
Purpura both lower legs upto thigh
and in both upper limbs -palpable ;confluent
Bp 110/80 mmHg
PR 86/min
CVS S1S2
RS NVBS
CNS NFND

PICTURE

P/A

Not distended
Moves with respiration
Flanks free
No distended veins
No visible peristalis
Soft
Tender
No organomegaly
No free fluid
BS heard

Past history: Patient admitted in GRH for the c/o


arthralgia and skin rashes 10 days ago
diagnosed as post viral
arthralgia ; treated and discharged
Not k/c/o DM; HT; PT
Personal history:
Not an alcoholic;smoker
Patient took over to medical ward

Provisional diagnosis
A/C abdominal pain with
purpura for evaluation

Pt managed with
IVF DNS 2 pints
Inj.Ciprofloxin 200 mg iv bd
Inj.Metronidazole 500 mg iv tds
Inj.Ranitidine 50 mg iv bd
Tab.Paracetamol 500mg tds

Differential diagnosis for medical causes of


pain abdomen
Diabetic ketoacidosis
Herpes zoster
Sickle cell anemia
A/c intermittent porphyria
Poly arteritis nodosa
Henoch schonelin purpura
Lead poisoning
Hyperlipidaemia
Allergic pain [C1 esterase
inhibitor deficiency]

Differential diagnosis for palpable purpura


1. Vasculitis
a.Cutaneous small vessel vasculitis
B.Polyartritis nodosa
C.Essential mixed cryoglobulinemia
2.Emboli
A.A/c menigococcemia
B.Disseminated gonococcal
infection
C.Rocky mountain spotted fever
D.Ecthyma gangrenosum

Urine
Albumintrace
Sugar
- nil
Deposit- 1-2pc/hpf
CBC
HB- 12.2GM/DL
TC-6400
DC P 65; L 32; E 3
ESR -8/12
PLC 345000
MCV 82.2
MCH 24.5
MCHC 31.2
PCV 38 %

RFT :
Urea -14
Creatine- 0.6
RBS 87
Na -132
K-3.7
Cl -94
Bicarbonate-18
ECG : Normal
CXR : Normal

USG abdomen : Normal except for probe


tenderness ; no appendicular mass
CT abdomen :

Peri appendicular edema seen


suggestion: a/c appendicitis

SP 3 09.02.11
CBC
Platelet count
TC
RBC
DC
PCV
MCV
MCH
MCHC
HB
ESR
Prothrombin time
APTT
INR

-424000
-12800 cells/ cumm
-5.51million/ul
P78; L12; E10
-45.7%
-32.9
- 26.9
-32.4
-14.8
-30mm/hr
14 sec
26 sec
1.0

BIOCHEMISTRY
Sugar
-87
Urea
21.8
Creatine 0.74
LIVER FUNCTION TEST
Total bilirubin
Direct bilirubin
AST
ALT
GGT
SAP
Total protein
Albumin
Globulin

-0.52
-0.20
-25 u/l
-17u/l
17u/l
- 160u/l
-7g/dl
4g/dl
-2.9 g/dl

ELECTROLYTES:
Na
-141
K
- 4.89
Cl
-103.4
SEROLOGY
HBSAG
Negative
ANTIHCV- Negative
HIV Negative
URINE MICROSCOPY
Leucocytes -3-4/hpf
Ep cells
- occassional
RBc
-2-3/hpf
SERUM LDH

219 u/l

Urine analysis 14.02.2011


Glu
-trace
Bil
-neg
Ketone neg
Sg 1.015
Blo
2+
ph
7.5
Pro
3+
Nit
neg
Leu
neg
STOOL Ova nil
Cyst nil
occult blood nil

IMMUNOLOGY

ANA 1:10 NEG


1:40
NEG
ASO
NEG
RF
NEG
CRP
NEG
C ANCA
NEG
P ANCA
NEG
Ig A
391mg/dl [ normal
80-350 mg/dl]

Histopathology report
Specimen:purpura right forearm biopsy
Microscopic description:stratified squamous
epithelium with hyperkeratosis ,
subepithelium shows congested blood vessels
infiltrated with lymphocytes
Impression : cutaneous small vessel vasculitis

DIAGNOSIS
HENOCH SCHONLEIN PURPURA
Patient treated with Tab. Prednisolone 1mg/kg
Tab.Ranitidine 150 mg bd
Tab.BCT tds

HSP is characterized by a classic tetrad of

purpura

Non thrombocytopenic palpable

Arthralgia or arthritis
Gastrointestinal and renal involvement
Rarely involvement of lungs ,
cns,genitourinary tract
Cutaneous involvement is most common
presentation
Also called anaphylactoid purpura

EPIDEMIOLOGY
90% of cases reported in children
Peak in children aged 4-7

Male:Female (1.5:1)
50% follow a URI
Renal disease is more severe in adults
Seasonal variation ; peak incidence in spring

PATHOGENESIS
Likely mechanism thought to be an immunecomplex mediated disease with deposits in
the glomerular capillaries, dermal capillaries
and GI tract.
Mesangial deposits of IgA are the same as those
seen in IgA nephropathy

PRECIPITATING ANTIGENS
INFECTIONS
URI
Measles
Rubella
Parvovirus B19
Mycoplasma
Coxsackie virus
Toxocara
Amebiasis
Salmonella

C.difficile
H.pylori
Adenovirus
Legionella
Tuberculosis
Mumps
Streptococcus
Morganella morganii

PRECIPITATING ANTIGENS
Drugs
Vancomycin
Streptokinase
Ranitidine
Cefuroxime
Diclofenac
Enalapril
Captopril

PRECIPITATING ANTIGENS
Other:
Food hypersensitivity
Cold exposure
Autosomal recessive Chronic granulomatous
disease
Myelodysplastic syndrome
Small cell lung cancer
Breast cancer

CLINICAL FEATURES
PALPABLE PURPURA: most commonly seen on

lower extremities and buttocks, however can


also been seen on the trunk and arms.
Lesions begin as erythematous macules and

progress to purpuric, non-blanching, nonpruritic


lesions that may become confluent

Gastrointestinal manifestations
One third with HSP have GI features
Colicky pain, nausea, vomiting, GI bleed
50% have occult bleeding
Major GI bleed in 5%
Intussusception 2%
Abdominal pain: sub-mucosal and intramural
extravasation of fluid and blood
Endoscopy: Erosive duodenitis

Renal manifestations

Abnormalities of urinary sediment


Acute nephritis
Progressive renal disease
Glomerular crescent formation - fatal outcome
Mortality 4%
Nephropathy adults 85%; children 25%
Renal insufficiency 13% in adults, 0% in children

HSP in Children & Adults


FEATURES

CHILDREN

ADULTS

Knees, ankles,
elbows, shoulders

Wrists, MCP,
PIP, MTP

Edema

Scalp, Face, Arms,


Hands, Feet Scrotal

Absent

Purpura

Limbs (usually
dependent areas)

Limbs, torso

Present

Present

Uncommon

Common

Course

Benign

More
fulminant

Familial

Rare

Absent

Arthritis/
Arthralgia

Abdominal pain
Renal
involvement

DIAGNOSTIC EVALUATION
May have mild leukocytosis
Normal platelet count
Occasionally eosinophilia
Normal serum complement levels
Elevated IgA in 50% cases

DIAGNOSIS
Generally a clinical diagnosis
Skin Biopsy: can be helpful and used to confirm
IgA and C3 deposits and leukocytoclastic
vasculitis.
Renal Biopsy: not usually needed for diagnosis.
Will show mesangial IgA deposits and
segmental glomerulonephritis

DIAGNOSTIC CRITERIA
ACR criteria-1990
three or more of the following cricteria
1.age 20 yrs or less at disease onset
2.palpable purpura
3.a/c abdominal pain with git bleeding
4.biopsy show granulocytes in the walls
of small arterioles and venules in superficial
layers of skin

EuLAR/PReS criteria -2006


mandatory criterion
1. palpable purpura
plus at least one of the following criteria
1.diffuse abdominal pain
2.iga deposition in any biopsy
3.arthritis or arthralgia
4.renal involvement[hematuria and or
proteinuria]

Chapell hill consensus group classification


system
primarily use nonclinical criteria
require only presence of small vesel
vasculitis with IGA deposition

Medications

Indication

Acetaminophen,
NSAIDs

Mild rash, arthritis

Oral steroids (1-2


mg/Kg)

Severe rash,
cutaneous edema,
severe colicky
abdominal pain,
scrotal and
testicular
involvement

IV steroids (1-2
mg/Kg)

Same as oral
Same as oral
steroids, should
steroids
be given if patient
is not able to
tolerate oral
medications

Comments

These cannot
prevent
development of
systemic
involvement but
can be helpful for
symptomatic
treatment.

High-dose IV
pulse steroids

Nephrotic range
proteinuria

High-dose IV
pulse steroids
plus
immunosuppressi
on

Rapidly
progressive
glomerulonephriti
s (RPGN),
hemorrhagic
involvement of
lungs, brain

Plasmapheresis
and/or IV
immunoglobulin
therapy

Refractory HSP to
combination
therapy (steroids
and
immunosuppressi
on), massive
hemorrhage in
gastrointestinal or
other organs

Decreases ESRD
progression (in
some case series
and reports)

Grade D
recommendation,
but evidence is
growing with
multiple case
series and
reports. This is
used as the last
resort to treat
refractory HSP.

MANAGEMENT
Usually self-limiting (1-6 weeks)
Steroids:
may decrease tissue edema, may aid in
arthralgias and some abdominal pain
Has not been shown to be beneficial in kidney
disease or dermal manifestations
Does not lessen chance of recurrence
Does not shorten duration of disease

Prognostic factors for HSP : .The worse prognostic factors:

(i) Greater than 8 years of age


(ii) Greater number of relapses
(iii) Higher creatinine level
at the onset
(iv) Proteinuria
greater than 1g/day
(v)
Hematuria, anemia at diagnosis
(vi) Development of hypertension
(vii) Membranoproliferative glomerulonephritis
(viii) Fever at presentation
(ix) Purpura above the waist
(x) Persistent purpura
(xi) Elevated
sedimentation rate.
(xii)
Elevated IgA concentration with reduced IgM( concentration
at the time of diagnosis.)
(xiii) Low factor XIII level

Ref:Harrison 17 e
Cmdt 2011
eMedicine