CASE CONFERENCEJuly 18, 2012

15 year old male with a rash

HISTORY

4 days PTC

Developed a red rash on the palms and solesIntensely itchyDiscomfort while walking

2 days PTC

(+) Mild throat discomfort(+) Low grade feverSought consult at the ED:Impression – Coxsackie Virus infectionTx: Diphenhydramine

Day of Admission

No relief from DiphenhydramineWorsening of the rashDifficulty in walking because of b/l ankle pain

History

Review of Systems

Denies vomiting, abdominal pain, changes in bowel habits, and changes in urine output

Past Medical History

Nodular acne; has been on Doxycycline 100 mg daily x 5 months

Family History Denies any medical/surgical problems among immediate family members

Social History Child lives in an apartment with parents and siblings. (+) Pets at home. No recent travel. HEAADDSS history non-contributory to the case

Physical ExaminationGeneral Appearance Alert and awake, not in distress.

Cooperative

Vital Signs Afebrile, 100/60, HR80; RR 20

Head, Eyes, Ears, Nose Throat, Neck

NCAT, pinkish conjunctivae, anicteric sclerae, nasal septum midline, TM’s intact, dry oral mucosa, non-hyperemic OP, supple neck, no CLAD

Chest and Cardiovascular CTAB, no wheezes, +S1/S2, no murmurs

Abdominal Exam Flat abdomen, normoactive bowel sounds, no tenderness to palpations, no CVA tenderness

Extremities No edema, no cyanosis, brisk capillary refill; No limitation in ROM

Neurologic Exam No focal neurologic findings; Gait difficulties

Physical Examination

ED Management

Concerns for vasculitis – Basic labs sent, which included coagulation panels

Strep infection partially ruled out with RST

Urinalysis

RPR, Rickettsial antibodies

ANA, RF

Patient booked for admission for observation

Laboratory Tests

CBCParameter Result

sNormal

WBC count 6.1 4.5-13.5

Hemoglobin

13.8 13-14.5

Hematocrit 41.1 36-43

Platelets 306 150-350

N 42

L 41

M 10

ChemistriesParameter

Results

Normal

Na+ 136 133-146

K+ 4.1 3.4-4.7

Cl- 106 98-107

Bicarb 28 20-28

BUN 7 5-18

Crea 0.6 0.5-1

Glucose 97 60-100

Calcium 9.3 8.6-10

Laboratory Tests

CHEMISTRIES OTHERS

Parameter Results

Normal

ALT 16 10-40

AST 20 15-45

Bilirubin 0.5 0.3-1.2

Albumin 3.8 3.2-5.1

Total Protein

7.3 6.0-7.9

Parameter Results

C3 126

C4 32

RPR Non-reactive

Rickettsial Negative

ANA Negative

RF Negative

Laboratory Tests

UrinalysisParameter Results

Color Yellow

Clarity Clear

SPG 1.029

pH 5.5

Proteins TR

Glucose Negative

Blood Negative

WBC 3/hpf

RBC 1/hpf

Sq Cells < 1/hpf

Henoch Schonlein PurpuraVincent Patrick Tiu Uy, MDPGY-2

History

Epidemiology

Peak age of onset: 3-15 years old

Exceedingly rare in the adult population

Males>Females

Very common during the cooler months and rare during the summer

Pathogenesis

Possible Etiologies

Upper Respiratory Tract Infections (~75%)

Streptococcal infections

Other infections

Vaccinations

Medications

Insect Bites

Clinical Manifestations

Rash of HSP

Arthritis and Arthralgias

Typically presents in 84% of patients with HSP, and is the presenting manifestation in 15% of the cases.

Oligoarticular (1-4 joints); Migratory; Mild

> Ankles/Knees

Usually no joint effusion and no swelling will be seen

Toddlers and younger children will refuse to ambulate

Does not cause permanent joint deformities

Gastrointestinal Symptoms

Can range from mild symptoms of nausea/vomiting and pain to significant events like bowel angina and GI bleeding.

Colicky pain

Massive GI hemorrhage is rare

Submucosal hemorrhage and bleeding Mesenteric vasculitis

Intussusception

Renal Disease

20-54% of cases; usually in patients with persistent rashes

Long-term outcome determined by extent of kidney involvement.

Most common presentation is nephritic syndrome with hematuria and mild/absence of proteinuria.

Nephrotic range proteinuria and altered kidney function tests predict a more progressive kidney disease

Watch out for high blood pressure – this may be a clue!

Refer to Renal

Findings on kidney punch biopsy = IgA nephropathy

Nephritic vs Nephrotic Syndrome

Nephritic Syndrome

Hematuria

Hypertension

Azotemia

Oliguria

Nephrotic Syndrome

24 hour urine protein >50 mg/kg/day

Low serum albumin

Hypertension

Hyperlipidemia

Other Presentations

Scrotal Pain

Central Nervous System

Peripheral Nervous System

Respiratory Tract

Eyes

Differential Diagnosis

Condition Presentation

AHEI 4 mos – 2 years; (+) Fever, purpura, ecchymosis and edema; Resolves spontaneously

Hypersensitivity Vasculitis

After drug exposure; Fever, urticaria, lymphadenopathy & arthralgias; Skin biopsy has no IgA

Rocky Mountain Spotted Fever

Presents with fever and rashes on the palms and soles; caused by insect bite

SLE Must satisfy 4/11 criteria for SLE

Meningococcemia Patient appears more septic; may be unvaccinated; Larger purpura and ecchymosis

ITP/HUS Platelet abnormalities are present

Reasons Behind Ancillary Procedures

Test Reason

Complete Blood Count Check platelets; anemia/bleeding

Coagulation Studies Bleeding disorders/Coagulopathy

Urinalysis Check for hematuria/proteinuria

Serum Creatinine Should be obtained if urinalysis is abnormal; always obtain in older patients

Abdominal Ultrasound (+) Severe abdominal pain

Skin Biopsy Usually not necessary unless manifestations are unclear

SUPPORTIVE Treatment of HSP

Most patients may be treated on an outpatient basis

Advise patients to rest until symptoms wear off

Prognosis is generally good, especially if no renal involvement

STRICT Follow-up should be advised

Criteria for Hospitalization

1. Inability to maintain adequate hydration orally

2. Severe anemia requiring transfusion

3. Severe abdominal pain

4. Significant GI bleeding

5. Changes in mental status

6. Severe joint involvement limiting ability to move

7. Renal insufficiency, hypertension and nephrotic syndrome

SYMPTOMATIC Treatment of HSP

Pain control may be achieved with NSAIDS.

No studies that relate worsening of GI bleeding in patients given NSAIDS or cyclooxygenase inhibitors

May give Naproxen, Acetaminophen or Ibuprofen

Glucocorticoid use is controversial

May be considered in hospitalized patients, symptoms that are severe enough to prevent oral fluid intake or severe joint symptoms that prevent ambulation.

Not enough data to support that steroid provide rapid improvement

Follow-up

Weekly or bi-weekly

BP + UA for blood

Monthly BP + UA for

blood

2 monthsRecovery ~ 6 months

Bi -monthly BP + UA for

blood

12 months

Obtain SERUM CREATININE anytime if (+) abnormalities

HSP of the BRAIN leading to CONFUSION!As the expert in Pediatric Henoch-Schonlein Purpura in St. Barnabas Hospital, you are called to see a 10 year old female who presented with palpable purpura of the buttocks and legs with pain on both knees. The doctor was convinced that this is HSP – and she apparently sent for labs. Which of the following laboratory work-up will make the diagnosis of HSP stronger?

A.Complete Blood Count and Coagulation studies

B.CBC and Urinalysis

C.Urine Dipstick

D.Abdominal Ultrasound

E.Anti-Nuclear Antibodies

HSP of the BRAIN leading to BRAIN INFARCT!!!An otherwise healthy 15 year old male was seen in the ED for rashes, arthralgia and abdominal pain. A diagnosis of HSP was made and the ED attending booked him for admission. You are the admitting resident on the floor. Which of the following situation warrants admission?

A.A hemoglobin level of 12.0 mg/dL with nosebleed for 1 minute

B.Rash involving the face, upper trunk and groin in addition to the typical leg and buttock rash

C.Patient was not responding to acetaminophen

D.Blood pressure of 140/80 with no proteinuria on dipstick

E.Fever of 101.2F and positive Guaiac test

THANK YOU!

I would like to thank Dr. Pertubal and Dr. Bhopi for the H&P

& Dr. Shafaghi for her guidance while managing this case