CASE CONFERENCE July 18, 2012 15 year old male with a rash 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 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 noncontributory to the case Physical Examination General 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 Differentials? 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 CBC Chemistries Parameter Results Parameter Results WBC count 6.1 Na+ 136 Hemoglobin 13.8 K+ 4.1 Hematocrit 41.1 Cl- 106 Platelets 306 CO2 28 N 42 BUN 7 L 41 Crea 0.6 M 10 Glucose 97 Calcium 9.3 Laboratory Tests CHEMISTRIES OTHERS Parameter Results Parameter Results ALT 16 C3 126 AST 20 C4 32 Bilirubin 0.5 RPR Non-reactive Albumin 3.8 Rickettsial Negative Total Protein 7.3 ANA Negative RF Negative Laboratory Tests Urinalysis Parameter 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 Purpura Vincent Patrick Tiu Uy, MD PGY-2 Epidemiology Peak age of onset: 3-15 years old Very common during the cooler months and rare during the summer Pathogenesis Possible Etiologies Upper Respiratory Tract Infections Streptococcal infections Other infections Vaccinations 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 Usually no joint effusion and no swelling will be seen Toddlers and younger children will refuse to ambulate Does not cause joint sequela Gastrointestinal Symptoms Can range from mild symptoms of nausea/vomiting and pain to significant events like bowel angina and GI bleeding. Massive GI hemorrhage is rare Submucosal hemorrhage and bleeding. Intussusception Renal Disease 20-54% of cases 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! Findings on kidney punch biopsy = IgA nephropathy 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 Recovery Weekly or bi-weekly BP + UA for blood Monthly BP + UA for blood Bi -monthly BP + UA for blood 2 months ~ 6 months 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