Henoch-scholen purpura

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OMDURMAN MILITARY HOSPITAL Unit of Dr: IBRAHIM MOHAMED IBRAHI Presented By: Dr. Manal M. Taha Case presentation

Personal data:- : 

Personal data:- Name : M. A.I Age : 4years Sex : female Residence : Al Feeha Tribe : Gallia Informant : mother & D.O.A : 03/08/2010

C/O : 

C/O Cough / 1wk Fever/1 wk Abdominal pain / 2days Joint pain and lower limbs swelling / 2days Skin rash / 2days

HPI:- : 

HPI:- The condition started 1 week P.T.A with dry cough the cough occur all the day not associated with S.O.B or vomiting. Also the condition associated with fever on and off relive by paracetamol . the mother consult a doctor and diagnose as having URTI and receive oral antibiotic and improve.

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Then 5days later she develop abdominal pain mainly around umbilicus colicky in nature ,on and off increase by eating associated with nausea and vomiting ,not contains bile or blood no diarrhea .Normal bowel habits no meleana or abdominal distention.

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She Also develop joint pain and swelling of the lower limbs mainly the ankle joint and dorsum of the feet . The pain is aggravating by movement , vague in nature with no reliving factor. The swelling is mainly on the dorsum of the feet.

During this 2days the mother notice that there is skin rash started in the lower limbs red in colour small in size she think its a mosquito bite but it become large in size and number and involve the both upper limbs it is itchy and become dark red.

Systemic review:- : 

Systemic review:- CPS NAD UGT :- urine normal in colour, amount ,frequency and no burning mictursion. CNS :- there is headache,no convulsions, change in sleep pattern or deterioration of consciousness.

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PMH : No history of similar condition , ecchymosis due to minor trauma or bleeding . No PH of admission, blood transfusion or drug ingestion. No PH of anemia ,joint pain or renal disease.

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Pregnancy & birth history: Prenatal, natal and post natal uneventfully. Developmental History : Passed through normal mile stones.

Nutritional history : Satisfactory. Vaccination: Fully vaccinated according to old E.P.I

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35 yrs 49 yrs 6yrs Family history: No FH of similar condition. There is FH of DM (her grand father), No FH of anemia ,arthritis ,bleeding disorders, or other connective tissue disorders . 8yrs 2yrs

Social history : They live in their on house composed of two rooms and one hall. there is water and electricity supply. They are of moderate socio-economic status .The disease has not impact on the family socially and financially.

Drug history : Not on long term medications. Not known to be hypersensitive to any drug.

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O / E pt looks unwell, not jaundiced ,pale or cyanosed, no dysmorphic features, afebrile. {show}

Vital signs PR 110 /min RR 20/min Temp 37.5C BP 100/60 mmHg

Anthropometric measurements Ht : 105 cm (50th percentile) Weight : 18 kg ( 75th percentile ) HC : 52cm (50th percentile)

Head & neck: : 

Head & neck: Eye :- no conjuctival hemorrhages , or cataract . Neck :- thyroid not enlarged , no palpable lymph nodes. Mouth :- buccal mucosa is normal in colour and there is no petechiae,gum bleeding telanjectasea or ulcers . Pharynex: no erythema or petechiae

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Hands examination : No petechial or purpuric rash or necrotic lesions. there is no clubbing

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Chest :- No skin rash, normal breath sounds. CVS :- Apex is in the 5th ICS, 1st and 2nd HS are normal.

Abdominal Examination: : 

Abdominal Examination: Abdomen not distended .no dilated superficial veins, surgical scars, skin rash or cautery marks. Umbilicus is inverted. hernial orifices are intact .no scratch marks There is mild tenderness ,no muscle guarding and no palpable masses . Liver and spleen are not enlarged, liver span is 10cm . kidneys not bimanually palpable. no shifting dullness. abdominal girth

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CNS :-pt is fully conscious, GCS 15 .all cranial nerves are intact . Posture, tone, power, reflexes and sensation in the Upper and lower limbs are normal.

skin Examination: : 

skin Examination: There is maculopabular and pinpoint rash red, purple, and brown in colour in the upper and lower limbs with sparing of the face, trunk ,palm and sole .its not tender ,itchy or blanching .there's no excoriation with normal skin turger and no dryness. (show)

musculoskelital Examination: : 

musculoskelital Examination: Joints examination :- Neck :- no tenderness normal flexion , extension ,lateral flexion and rotation Shoulders ex :- no swelling or tenderness and normal flexion ,abduction ,adduction ,external rotation ,internal rotation and extension

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Elbows ex :- no deformity , swelling or tenderness normal flexion, extension , supination and pronation. Wrists ex :- no swelling ,deformity normal flexion ,extension ,radial deviation ,ulnar deviation . MCP joints :- normal flexion and extension .

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Hips ex :- in extension position no tenderness normal flexion ,internal rotation ,external rotation ,abduction and adduction . Knees joint ex :- no muscle atrophy or hypertrophy no swelling or tenderness .flexion ,extension .

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Ankles joint :- there is swelling and tenderness .plantar flexion and extension difficult to be assess due to pain . There is non pitting oedema in the dorthum of the feet . ex difficult to assess due to pain . Other joints examination were normal. No muscle atrophy or hypertrophy .

summary : 

summary 4 yrs old girl presented with abd pain , joint pain and swellig and skin rash . Preceded by URTI.with no history of arthritis or bleeding disorder . There is palpable non blenching purpuric and peteichial rash in the both upper an lower limbs with unremarkable abd exa finding .

Investigations : 

Investigations Urine general : normal in colour no RBCS or granular cast Stool general : Normal. Stool for occult blood (+ve) CRP: 8mg/(+ve) .

CBC : 

CBC

RFT : 

RFT

LFT:- : 

LFT:-

Bleeding profile : 

Bleeding profile

Abdominal U/S : 

Abdominal U/S liver is normal in size, with normal texture and echogenecity no focal lesion seen. normal portal and hepatic veins. Spleen not enlarged with normal texture and vein calibre. Normal pancreas. No enlarged LN. GB: normal no stones. No intra or extra biliary duct dilatation. normal kidneys shadow. Normal urinary bladder. No ascites. Large and small bowel are normal with no sign of obstruction or intussusception's

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Immunology Antinuclear Ab :- --Ve A.S.O titre :- <200 IU /L Rheumatoid factor :- -Ve . d.DNA :- --Ve.

Diagnosis : Henoch-scholen purpura

. NSAI drugs Predinosolone tab Follow up Management & Follow -up:-

Literature review

Henoch-Scholein Purpura : 

Henoch-Scholein Purpura Henoch-Schonlein purpura (HSP) is a self-limited, systemic, nongranulomatous, autoimmune complex, small vessel vasculitis, with multiorgan involvement.

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The annual incidence varies geographically from 6.2 to 70.3 per 100,000 in children less than 17 years of age with slight male predominance (M:F = 1.2:1.0). Peak age incidence is 4–years and 90% of HSP cases occur before the age of 10 years. Worldwide, Afro-Caribbeans have the least incidence

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HSP is characterized by a classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement, and rarely, other systems (lungs, central nervous system, genitourinary tract) . Cutaneous involvement is the most common presentation, although patients may present with involvement of other organ systems

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Two major classification systems are used to make a diagnosis of HSP. The first, from the American College of Rheumatology, requires 2 or more of the following to be present: Patient age younger than 20 years Palpable purpura Abdominal pain or GI bleeding Extra vascular or per vascular granulocytes on biopsy The second classification system, from the Chapel Hill Consensus Group, primarily uses nonclinical criteria, and requires only the presence of small-vessel vasculitis with IgA deposition.

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In general, most of the HSP cases are self-limited, with good prognosis and five-year survival rates of 95% . One third of the patients have relapses, which are milder and shorter in duration, usually within 4 months, and involving the same organs

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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

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