R1 林威竹 Case from 岑秋良醫師 Pediatric Case conference

Patient ’ s Profile 姓名：王 X 性別：男 出生日： 檢查日期： 07/12/2007 （四） 年齡： 8 歲四個月 TPR:37.5/ ℃ P:82/min R:22/min BP: 103/64/mmHg BW: 35 kg BH: 140 cm

Chief complain bilateral lower leg skin rash and joint pain on four extremities for 1 day( since 7/11) Right side 3rd DIP swelling was noted and then following left 2nd DIP, bilateral knee joint swelling accompanied by ankle joint pain

Present Illness BIL LOW LEGS SKIN RASH FOR ONE DAY ( SINCE 2007/7/11) BIL KNEE AND HAND PAIN WITH SWELLING BIL LEG EDEMA NO TRAUMA NO DIZZINESS NO ABD PAIN NO COUGH BUT MILD RN/ NO SORE THROAT NO HEADACHE NO V/D TRAVEL HISTORY: 墾丁 ( 2 days ago travel with swimming)

Physical Examination General appearance : weak Conscious : clear E4 V5 M6 Pupil size : 3+/3+ HEENT: not anemic not icteric no lymphadenopathy NECK:SUPPLE Chest: BS : clear HS : RHB, no murmur Abdomen: soft and flat no tenderness no rebound pain Extremities:joint pain on four extremities Pitting edema:(+/-) Free movement:(+)

skin image Left Ankle Left Leg Right Hand Right Leg

Past history Drug allergy ： NKA Birth History ： 2800gm Term baby of G1 P1 mother by C/S Admission history: acute gastroenteritis on 2006/12/ /01/02 Vaccine as schedule

Initial Impression Other nonthrombocytopenic purpuras  HSP

Initial Order Chest A-P View(Supine) CBC/DC, PT, PTT BC No steroid was administrated in ER

Radiography of Chest A-P View(Supine) Show: No definite lung infiltration or consolidation. Mild cardiomegaly The bony alignment is normal 報告日期： 07/13/

Laboratory Data 檢驗項目 檢驗值 單位 H/L 參考值 改 WBC /uL M F RBC 5.11 million/uL M F Hemoglobin 14.3 g/dL M F12-16 Hematocrit 41.5 % M41-53 F36-46 MCV 81.2 fL MCH 28.0 pg/Cell MCHC 34.5 g/dL RDW 12.2 % Platelets /uL Segment 61.0 % Lymphocyte 29.1 % Monocyte 7.0 % 0-12 Eosinophil 2.7 % 0-5 Basophil 0.2 % 0-1

檢驗項目 檢驗值 單位 H/L 參考值 改 P.T 13.3 sec (INR <1.2,Pre ve.: Nor.plasma mean 11.3 sec Treat of Venous Thr ombus INR 1.2 Treat of Arterial D is ) APTT 29.2 sec (Therapeut ic x Nor.plasma mean 28.0 sec Plasma mean of APTT

檢驗項目 檢驗值 單位 H/L 參考值 Sugar 104 mg/dL H (child) BUN (B) 10 mg/dL 5-20 (child) Creatinine(B) 0.5 mg/dL (infant-18Y) Total Bilirubin 0.4 mg/dL (>5d-18y) AST (GOT) 23 U/L (2-18Y) Na 138 meq/L (<18Y) K 4.0 meq/L (<18Y) CRP mg/L H < 5

B/C No Growth for Aerobes and Anaerobes

The Following Hospitalization Course Admission on 2007/7/13 Impression: HSP Plan: 1.Supportive care 2.Sympyomatic treatment 7/17: Mild leucocytosis was developed during this admission WBC: /ul and Seg: 75.3 % lymphocyte: 18.1 % with Hb: 13.1 g/dl and ESR: 0 7/19: Gr. A Strep Ag Negative 7/20: 血清病毒檢驗 : all negative or within normal limit (RF, IgG. IgA. IgM. IgE,C3. C4, ANCA) Steroid was administrated during this admission No GI S/S or Renal involvment was noted during this admission

HSP Discussion

INTRODUCTION Henoch-Schönlein purpura (HSP) is the most common form of systemic vasculitis in children HSP is self-limited in the great majority of cases The disease is characterized by a tetrad of clinical manifestations, vary in their occurrence and order of presentation # Palpable purpura in patients with neither thrombocytopenia nor coagulopathy # Arthritis/arthralgia # Abdominal pain # Renal disease

PATHOGENESIS HSP is an immune-mediated vasculitis associated with immunoglobulin A (IgA) deposition Although a variety of infectious and chemical triggers have been proposed, the underlying cause of HSP remains unknown The characteristic finding of HSP is leukocytoclastic vasculitis accompanied by IgA immune complexes within affected organs involvement of small vessels (primarily postcapillary venules) within the papillary dermis

CLINICAL MANIFESTATIONS The classic tetrad of HSP includes: # Palpable purpura without thrombocytopenia and coagulopathy (all) # Arthritis/arthralgia (Taiwan: 43%, 75%) # Abdominal pain (50%, 20~30% GI bleeding) # Renal disease (21~ 54%)

These clinical manifestations may develop over the course of days to weeks and may vary in their order of presentation Purpura and joint pain are usually the presenting symptoms but this is not always the case In the absence of the classic purpuric rash, the diagnosis of HSP may not be obvious initially Patients who present with significant joint or abdominal symptoms without the skin manifestations may be thought to have an infectious or surgical process

Skin manifestations The classic rash of HSP is not the initial presenting sign in about one-quarter of affected children As a result, it may be difficult to make the diagnosis of HSP prior to its development in patients who present with other clinical manifestations such as abdominal pain or arthritis

The rash often begins with erythematous, macular, or urticarial wheals The wheals then coalesce and evolve into the typical ecchymoses, petechiae, and palpable purpura The rash typically appears in crops, in a symmetrical distribution, and located in gravity/pressure-dependent areas such as the lower extremities. The buttocks are often involved in toddlers, and the face, trunk, and upper extremities in nonambulatory children Localized subcutaneous edema is a common feature that may be found in dependent and periorbital areas, especially in younger children (<3 years of age).

Purpura D/D Petechiae and purpuric rashes may be associated with septicemia, idiopathic thrombocytopenic purpura, hemolytic uremic syndrome, leukemia, and coagulopathies (eg, hemophilia). Platelet count and coagulation studies differentiate HSP from these entities There are several conditions that can present with purpura with normal platelet counts and coagulation studies such as Acute hemorrhagic edema of infancy (AHEI), Hypersensitivity vasculitis, and Other small vessel vasculitis

Acute hemorrhagic edema of infancy (AHEI) Biopsy of the skin demonstrates a leukocytoclastic vasculitis with occasional IgA deposition children between the ages of 4 months to 2 years Involvement of the kidney and the gastrointestinal tract is uncommon

Hypersensitivity vasculitis Biopsy: IgA deposition is absent History: after exposure to drugs or infection, or without an identifiable trigger S/S: Patients present with fever, urticaria, lymphadenopathy, and arthralgias, but not usually glomerulonephritis

Other small vessel vasculitis There are a number of causes of small vessel vasculitis (including HSP) that may present with asymmetric polyneuropathy, palpable purpura, and/or pulmonary-renal involvement primary vasculitis or conditions secondary to a connective tissue disorder or infectious disease In general, these diseases, which mimic HSP, are uncommon in children

DIAGNOSIS The diagnosis of HSP is usually based upon clinical manifestations of the disease. The diagnosis is straightforward when patients present with the classic signs and symptoms, especially palpable purpura of the lower extremities and buttocks. In patients with incomplete or unusual presentations, a biopsy of an affected organ (eg, skin or kidney) that demonstrates leukocytoclastic vasculitis with a predominance of IgA deposition confirms the diagnosis of HSP

Biopsy In pediatric patients, biopsy is reserved for patients with an unusual presentation of HSP (ie, no rash, or an atypical rash) or those with significant renal disease

Laboratory tests No laboratory test is diagnostic for HSP. Serum IgA levels have been reported to be elevated in 50 to 70 percent of patients with HS Findings on routine blood tests (eg, complete blood cell count, serum chemistries, and urinalysis) are non-specific. Results generally reflect the triggering condition Patients may have a normochromic anemia because of occult or overt gastrointestinal bleeding

Demonstration of a normal platelet count and coagulation studies (prothrombin time) are imperative to distinguish HSP from other diseases that present with purpura on account of thrombocytopenia or coagulopathy. Urinalysis should be performed in all patients with HSP. In general, the findings reflect the degree of renal involvement and may include the presence of red or white cells, cellular casts, and proteinuria

Emergency Room Care Emergency department treatment is supportive, with frequent monitoring of vital signs. For minor complaints of arthritis, edema, fever or malaise, symptomatic treatment is advised, including use of acetaminophen, elevation of swollen extremities, eating a bland diet, and adequate hydration

Steroid(1) corticosteroids may be considered in the following serious situations: Persistent nephrotic syndrome Crescents in more than 50% of glomeruli Severe abdominal pain Substantial GI hemorrhage Severe soft tissue edema Severe scrotal edema Neurologic system involvement Intrapulmonary hemorrhage

Steroid(2) Therefore, in summary, it appears that if the patient is subject to glomerulonephritis, systemic steroids would be in order to protect the kidneys even though the occurrence of relapses may be more frequent. In contrast, if the patient has gastrointestinal pathology associated with his or her HSP, systemic steroids are not helpful and should be avoided

pediatric consensus criteria clinical practice in which a clinician is more likely to seek features to distinguish HSP from gastroenteritis or appendicitis than from Wegener's granulomatosis The criteria included palpable purpura without thrombocytopenia and coagulopathy as a mandatory finding and one or more of the following: # Diffuse abdominal pain # Arthritis or arthralgia # Any biopsy with predominant immunoglobulin A (IgA) deposition

Thanks for your attention!