1. CASE CONFERENCE July 18, 2012

2. 15 year old male with a rash

3. HISTORY 4 days PTC Developed a red rash on the palms and soles Intensely itchy Discomfort while walking 2 days PTC (+) Mild throat discomfort (+) Low grade fever Sought consult at the ED: Impression – Coxsackie Virus infection Tx: Diphenhydramine Day of Admission No relief from Diphenhydramine Worsening of the rash Difficulty in walking because of b/l ankle pain

4. 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

5. Physical Examination General AppearanceAlert and awake, not in distress. Cooperative Vital SignsAfebrile, 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 CardiovascularCTAB, no wheezes, +S1/S2, no murmurs Abdominal ExamFlat abdomen, normoactive bowel sounds, no tenderness to palpations, no CVA tenderness ExtremitiesNo edema, no cyanosis, brisk capillary refill; No limitation in ROM Neurologic ExamNo focal neurologic findings; Gait difficulties

6. Physical Examination

7. 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

8. Laboratory Tests CBC ParameterResult s Normal WBC count6.14.5-13.5 Hemoglobi n 13.813-14.5 Hematocrit41.136-43 Platelets306150-350 N42 L41 M10 Chemistries Paramete r Result s Normal Na+136133-146 K+4.13.4-4.7 Cl-10698-107 Bicarb2820-28 BUN75-18 Crea0.60.5-1 Glucose9760-100 Calcium9.38.6-10

9. Laboratory Tests CHEMISTRIESOTHERS ParameterResult s Normal ALT1610-40 AST2015-45 Bilirubin0.50.3-1.2 Albumin3.83.2-5.1 Total Protein 7.36.0-7.9 ParameterResults C3126 C432 RPRNon-reactive RickettsialNegative ANANegative RFNegative

10. Laboratory Tests Urinalysis ParameterResults ColorYellow ClarityClear SPG1.029 pH5.5 ProteinsTR GlucoseNegative BloodNegative WBC3/hpf RBC1/hpf Sq Cells< 1/hpf

11. Henoch Schonlein Purpura Vincent Patrick Tiu Uy, MD PGY-2

12. History

13. 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

14. Pathogenesis

15. Possible Etiologies  Upper Respiratory Tract Infections (~75%)  Streptococcal infections  Other infections  Vaccinations  Medications  Insect Bites

16. Clinical Manifestations

17. Rash of HSP

19. 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

20. 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

22. 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

23. 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

25. Other Presentations  Scrotal Pain  Central Nervous System  Peripheral Nervous System  Respiratory Tract  Eyes

26. Differential Diagnosis ConditionPresentation AHEI4 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 SLEMust satisfy 4/11 criteria for SLE MeningococcemiaPatient appears more septic; may be unvaccinated; Larger purpura and ecchymosis ITP/HUSPlatelet abnormalities are present

27. Reasons Behind Ancillary Procedures TestReason Complete Blood CountCheck platelets; anemia/bleeding Coagulation StudiesBleeding disorders/Coagulopathy UrinalysisCheck for hematuria/proteinuria Serum CreatinineShould be obtained if urinalysis is abnormal; always obtain in older patients Abdominal Ultrasound(+) Severe abdominal pain Skin BiopsyUsually not necessary unless manifestations are unclear

28. 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

29. 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

30. 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

31. 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

32. 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

33. 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

34. 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