Henoch Schonlein Purpura (HSP) is an inflammatory vasculitis disorder that mainly affects children aged 3-15 years old. It is characterized by a generalized vasculitis of small vessels in the skin, GI tract, kidneys, joints, and rarely lungs and CNS. The cause is unknown but likely multifactorial involving genetic, environmental, and antigenic factors. Common symptoms include a rash, abdominal pain, arthritis or arthralgia, and potential renal involvement seen as hematuria and/or proteinuria. Diagnosis is usually made clinically based on seeing a palpable purpura rash along with at least one other symptom. Treatment focuses on pain management and supportive care as the

1. Henoch Schonlein Purpura Ahmad Abid Abas 2

2. What is HSP? Anaphylactoid purpura,Purpura rheumatica, Leukocytoclastic vasculitis. It is an inflammatory disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS. Mainly affects children 3-15 yr age.Rarely below 2 yr age.

3. Etiopathogenesis Unknown etiology. Multifactorial with genetic, environmental and antigenic components. More than 75% of patients report antecedent upper-respiratory, pharyngeal, or GI infections. An immunoglobulin A (IgA)–mediated autoimmune phenomenon. (clinical manifestation result fr this Ag-Ab complex)

4. How to Approach ? c/o – Usually the rash is the first to appear and the child is brought to medical attention by his/her parents. Hx – Prodromal symptoms such as headache,fever,anorexia. O/E – Rash --> Morphology (palpable) ?,(What type cutaneous lesions (petichea,ecchymosis)?,Site of distribution (thighs,buttocks) ?

5. How to Approach ? Other clinical features to be considered : 1) Abdomen – crampy abdominal pain. 2) Joints – migratory arthralgia,arthritis in ankles,knees (big joints) – expect periarticular swelling + significant pain. 3) Renal – (HTN,edema,oliguria and hematuria +/- azotemia) similar CP of APSGN.

6. How to Approach ? Dx - Palpable purpura (mandatory) in the presence of at least one of the following four features: • Abdominal pain • Arthritis (acute) or arthralgia • Renal involvement (any haematuria and/or proteinuria) • Any biopsy showing predominant Iga deposition

7. What to be expected in Investigations? Electrolyte values  Normal unless excess vomit. BUN/creatinine(RFT) – May ↑ in presence renal involvement. CBC – (exclude thrombocytopenia) leukocytosis possibly eosinophilia,Platelet counts N or ↑ .Hb or Ht values may be N or ↓ 2ry to bleeding. Urinalysis – ( hematuria, proteinuria, and occasional red cell casts.) (like APSGN)

8. How to Approach ? Criteria ITP Acute Leukimia Aplastic Anemia HSP Hb/RBC count Normal ↓ ↓ Normal WBC count Normal ↑↑↑ ↓ ↑ in some cases. Platelet count ↓ ↓ ↓ Normal

9. How to narrow DDx ? Tourniquet test [Hge tendency,capillary fragility] Antistreptolysin-O (ASO) titer [recent Strept. infection] Monospot test [rapid test for Mono. (EBV)] Antinuclear antibody (ANA) test [exclude SLE] Rheumatoid factor (RF) test [exclude RA] Determination of C3/C4 levels (diff. fr APSGN) Prothrombin time (PT) and activated partial thromboplastin time (aPTT) Blood cultures [potential infection,meningitis) Abd U/S – [Evaluate Intussusception & exclude appendicitis]

10. How to Manage ? Rx is largely supportive. Abdominal pain – Common and self-limited. Joint pain – NSAID agents Cutaneous manifest. – self-limited may relapse. Ch. Cutaneous lesion – colchicine,aspirin,systemic CST. No optimal management for GIT and Renal involvement.Should be follow up closely.

11. Conclusion (clinical point of view) HSP is diagnosed clinically. GI involvement is early symptom and renal disease is late symptom. If occur in adults , it is more severe. Palpable purpura,abd pain and arthritis is clinical characteristic triad of HSP in pediatric.

12. References 1) The Indian Academy of Pediatrics,Bulletin of Rheumatology Chapter IAP September 2010. 2)http://emedicine.medscape.com/article/804681-clinical 3) http://www.orpha.net/data/patho/GB/uk-HSP1.pdf