PROF.DR.NOORUL AMEENDR.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 o

CVS S1S2

RS NVBS

P/A tenderness in right iliac fossa

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; PTPersonal history: Not an alcoholic;smokerPatient 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 Albumin- trace

Sugar - nil Deposit- 1-2pc/hpfCBC 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 09.02.11CBC Platelet count -424000 TC -12800 cells/ cumm RBC -5.51million/ul DC – P78; L12; E10 PCV -45.7% MCV -32.9 MCH - 26.9 MCHC -32.4 HB -14.8 ESR -30mm/hr

Prothrombin time 14 sec APTT 26 secINR 1.0

BIOCHEMISTRY

Sugar -87 Urea – 21.8 Creatine – 0.74

LIVER FUNCTION TEST

Total bilirubin -0.52 Direct bilirubin -0.20 AST -25 u/l ALT -17u/l GGT – 17u/l SAP - 160u/l Total protein -7g/dl Albumin – 4g/dl Globulin -2.9 g/dl

ELECTROLYTES: Na -141 K - 4.89 Cl -103.4SEROLOGY 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 reportSpecimen:purpura right forearm biopsyMicroscopic 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

Non thrombocytopenic palpable purpura

Arthralgia or arthritis

Gastrointestinal and renal involvement

Rarely involvement of lungs , cns,genitourinary tract

Cutaneous involvement is most common presentation

Also called anaphylactoid purpura

EPIDEMIOLOGY90% 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

PATHOGENESISLikely mechanism thought to be an immune-

complex 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

INFECTIONSURIMeaslesRubellaParvovirus B19MycoplasmaCoxsackie virusToxocaraAmebiasisSalmonella

C.difficileC.difficile

H.pyloriH.pylori

AdenovirusAdenovirus

LegionellaLegionella

TuberculosisTuberculosis

MumpsMumps

StreptococcusStreptococcus

Morganella Morganella morganiimorganii

PRECIPITATING ANTIGENSDrugs

VancomycinStreptokinaseRanitidineCefuroximeDiclofenacEnalaprilCaptopril

PRECIPITATING ANTIGENSOther:

Food hypersensitivityCold exposureAutosomal recessive Chronic granulomatous

diseaseMyelodysplastic syndromeSmall cell lung cancerBreast cancer

CLINICAL FEATURESPALPABLE 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 manifestationsOne third with HSP have GI featuresColicky pain, nausea, vomiting, GI bleed50% have occult bleedingMajor GI bleed in 5% Intussusception 2%Abdominal pain: sub-mucosal and intramural

extravasation of fluid and bloodEndoscopy: Erosive duodenitis

Renal manifestationsAbnormalities 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 & AdultsFEATURES CHILDREN ADULTS

Arthritis/ Arthralgia

Knees, ankles, elbows, shoulders

Wrists, MCP, PIP, MTP

Edema Scalp, Face, Arms, Hands, Feet Scrotal

Absent

Purpura Limbs (usually dependent areas)

Limbs, torso

Abdominal pain Present Present

Renal involvement

Uncommon Common

Course Benign More fulminant

Familial Rare Absent

DIAGNOSTIC EVALUATIONMay have mild leukocytosis

Normal platelet count

Occasionally eosinophilia

Normal serum complement levels

Elevated IgA in 50% cases

DIAGNOSISGenerally 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 Comments

Acetaminophen, NSAIDs

Mild rash, arthritis

Oral steroids (1-2 mg/Kg)

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

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

IV steroids (1-2 mg/Kg)

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

Same as oral steroids

High-dose IV pulse steroids

Nephrotic range proteinuria

Decreases ESRD progression (in some case series and reports)

High-dose IV pulse steroids plus immunosuppression

Rapidly progressive glomerulonephritis (RPGN), hemorrhagic involvement of lungs, brain

Plasmapheresis and/or IV immunoglobulin therapy

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

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

MANAGEMENTUsually 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 recurrenceDoes 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