Morning Report

D Spencer Mangum, PGY3 10/28/13

Presentation
5 yo previously healthy male, presenting with: Intermittent fever x12-13 days (Tmax 103) Myalgias / Arthralgia x4 days
Initially bad enough that he had to be carried from

school to the car. Improved prior to presentation.

Significant Emesis (NB/NB) with decreased PO

x2 days Mild erythematous rash to palms and soles x1 day No known sick contacts, but attends daycare.

Presentation
ROS: No conjunctivitis, cervical lymphadenopathy, red lips,

red tongue, or diarrhea. PMHx: Previously healthy, no surgeries. No regular medications. Imms UTD. NKDA. Normal development. FHx: No significant childhood disease. SHx: Lives in Utah with his mother and 2 half siblings (parents divorced) VS:
T 38.6, HR: 108, BP: 139/71, RR: 20. O2 Sat >95% RA

EXAM:
Notable positives: Mild distress, dry lips & tongue, scattered 1-

3 mm erythematous macules (blanching) on palms and soles Notable negatives: No LAD (cervical or inguinal), No murmurs / well perfused, abd soft NT/ND, no hepatosplenomegaly, no peripheral edema

Presentation
Seen in the ED where he was felt to be

hypovolemic secondary to his emesis / poor PO, given 40 ml/kg NS and admitted to the floor.
Pt also began complaining of abdominal pain while

in the ED. KUB had a nonobstructive bowel gas pattern with moderate stool burden.
After admission, pt began having episodic,

significant, crampy, periumbilical abdominal pain

Would draw legs up to chest with pain Pt appeared normal in between episodes

Differential Diagnosis?
Viral:
Enterovirus Adenovirus Coxsackie EBV

Endocarditis
Atypical Kawasaki HSP
Intussusception
Vasculitis NOS

Hospital Course
Abdominal U/S on DOH 1 revealed a Mid-abdominal

small bowel-small bowel intussusception General surgery was consulted and pt underwent a exploratory laparoscopy with a 3 cm small bowel resection that same day. Pt eventually started on Amlodipine for continuing HTN (Initial U/As without proteinuria / hematuria) Pt discharged to home 1 week later with no clear unifying diagnosis After discharge, pt developed classic HSP purpuric rash and IgA staining of his small bowel came back positive confirming the diagnosis
Later U/As also had microscopic hematuria / proteinuria

consistent with HSP renal involvement

Henoch-Schnlein purpura (HSP)

Underlying cause is unknown, but it is an immune-

mediated vasculitis associated with immunoglobulin A deposition (aka, IgA vasculitis)

Possible infectious trigger? Occurs between ages 3-15 yo (peak ages 4-6)

Characterized by 4 main symptoms

Can develop in any order and at any time over a period

of several days to several weeks 1. Palpable purpura

Usually the presenting symptom, although can have delayed

onset in ~25% of children Rash is located in gravity/pressure-dependent areas such as the lower extremities / buttocks Without thrombocytopenia / coagulopathy
2.

Arthritis / arthralgia
Usually transient without any joint erythema / edema. Does not

have any chronic sequelae.

Palpable Purpura:

http://cvandir.blogspot.com/2012/11/the-vasculitides-henoch-schonlein.html

Henoch-Schnlein purpura (HSP)

(4 main symptoms continued)
3.

Abdominal Pain
Caused by submucosal hemorrhage and edema Intussusception is the most common GI complication (~3.5% of HSP

patients) Ileoileal location versus Ileocolic o Contrast enemas cannot detect ileoileal intussusception typically seen in HSP!

4.

Renal Disease
Pathologically identical to IgA Nephropathy Among patients with renal disease

80% have hematuria (predominantly microscopic) 50% have proteinuria 20% had nephrotic range proteinuria with or without edema or hypoalbuminemia, an elevated BUN or creatinine, and/or hypertension Short-term outcome of renal disease in HSP is favorable for most patients, but a minority of patients with HSP have persistent renal manifestations

Henoch-Schnlein purpura (HSP)

Diagnosis: Typically made clinically With unusual presentations (ie, no / atypical rash) a biopsy can confirm the diagnosis
Typically skin is biopsied, but in cases of significant renal

involvement the kidney may be biopsied instead A predominance of IgA deposition confirms the diagnosis of HSP
No labs confirm the diagnosis, but a U/A (+/- on BUN /

Cr) should be checked if diagnosis suspected Abdominal ultrasound can detect increased bowel wall thickness, hematomas, peritoneal fluid, and intussusception

Henoch-Schnlein purpura (HSP)

Treatment: Ideally occurs in the outpatient setting with primarily supportive care (adequate hydration, rest, and symptomatic relief of pain Ibuprofen / motrin). Hospitalization typically occurs if pt is unable to:
Severe abdominal pain Typically treated with NSAIDs (Naprosyn) and Steroids, although this is controversial May need to be IV given poor absorption if bowel wall edema is present Avoid NSAIDs if significant renal involvement and or GI bleeding Maintain adequate hydration requiring IV fluids Significant renal involvement / insufficiency requires

close monitoring and likely HTN control Significant GI bleeding

Henoch-Schnlein purpura (HSP)

Outcome: Overall excellent prognosis and disease is self limited in the great majority of cases 1/3 of patient will have recurrence, typically within 4 months of initial diagnosis with less severe symptoms HSP patients without renal involvement should be followed with U/A and BP monitoring every 1-2 weeks for 1-2 months after diagnosis to screen for the possible development of HSP nephritis. Patients with HSP nephritis should be followed by nephrology

Summary
5 yo male (peak age group) presenting with fever,

arthralgias, HTN, and vomiting / abdominal pain. Fever / initial rash (viral trigger of HSP?) Vomiting then significant abdominal pain (Intussusception / HSP) 2. Myalgias / Arthritis 3. HTN (later U/As consistent with renal involvement) 4. Classic Rash developed quite late in the patient
1.

Diagnosis confirmed by IgA staining