A Painful, Purpuric Rash Lydia Burland ST1 Paediatrics

Introduction Brief case study Common precipitants and presentation Initial investigation and management Indications for referral and follow up Key facts for future practice

Case Study: Presentation 16 year old boy Presented with a painful rash on both legs started less than 12 hours ago gradually spreading becoming increasingly painful now unable to weight bear

Case Study: Background HPC: 1st presented 2 days ago with sore throat & fever diagnosed with viral URTI discharged with analgesia PMH: normally fit and well DH: no allergies or regular medications

Case Study: Examination Obs stable and apyrexial Bilateral, non-blanching purpuric lesions Multiple blisters and bullae Up to the knees Extremely tender

http://www.healio.com/pediatrics/journals/pedann/%7Bd731347e-9464-4687-a5e6-99322b26a62b%7D/a-14-year-old-boy-with-bullous-lesions

Case Study: Investigations WCC 15.5 (8.4) Streptolysin O antibody 1310 (range <200) ANA/ANCA/RhF negative Throat swab negative Urine dipstick negative On presentation his WCC was raised, and had gone up since his prior admission 2 days earlier. He also had a high levels of streptolysin antibodies (the normal range being less than 200 in children and 400 in adults) which indicate past or present infection. Levels rise after 1-3 weeks of strep infection, peak at weeks 3-5 and fall back within normal range at around 6 months. All his other bloods were unremarkable and his throat swab and urine dip were negative.

Case Study: Initial Management Henoch-Schönlein Purpura 5/7 Penicillin V Regular paracetamol and NSAIDs Topical betnovate and hydromol cream Follow up in dermatology clinic in 10 days

Case Study: Further Progress Re-admitted 8 days later with; - Worsening, painful rash - Arthralgia - Abdominal pain Admitted under rheumatology Treated with IM depomedrone and reducing course of high dose prednisolone Rheumatology follow up in 1 week He was re-admitted under rheumatology 8 days later with worsening rash, which had spread to his upper limbs, arthralgia in his fingers, wrists, elbows, ankles and left knee and left-sided abdominal pain. He was treated with IM and high dose oral steroids and oral morphine before discharge with follow up in one week.

Henoch-Schönlein Purpura IgA mediated autoimmune vasculitis Predominantly affects children aged 3-10 yrs 20x less common in adults Often more severe disease in teenage/adult patients Henoch-Schonlein Purpura is an IgA mediated autoimmune vasculitis common in children aged 3 to 10 years. Females and Caucasians are at a very slightly increased risk. Though HSP is 20x less common in adults, it does present to adult services, especially in adolescent patients, and therefore it is important as acute adult physicians we are able to recognise the ways in which it presents, and what management needs to be initiated. Especially important is knowing which cases need further referral and follow up.

Henoch-Schönlein Purpura Recognised precipitants for HSP include; Infections, typically group A streptococcus, mycoplasma and Epstein-Barr virus Vaccinations Environmental exposure to certain allergens, pesticides and insect bites 50% have preceding URTI

Henoch-Schönlein Purpura Mild illness with fever Symmetrical erythematous macular rash Back of legs Buttocks Ulnar surface of arms Macules evolve into typical purpuric lesions These may coalesce, resembling bruises The majority of HSP cases present in the winter months, with 50% cases following an upper respiratory tract infection. Most patients present with mild illness and fever, and a symmetrical macular rash on the back of the legs, buttocks and ulnar surface of the arms. Over the course of 24 hours these macules then evolve into the typical purpuric lesions associated with HSP. They may coalesce to resemble extensive bruising. In adolescents these may then progress to bullae, which are much rarer in children.

Henoch-Schönlein Purpura 50% of cases have renal involvement 10% serious 1% progress to ESRF Rarer complications include; Intussusception GI bleeding MI Seizures Bowel infarction Mononeuropathies The main complication of HSP is renal involvement which occurs in 50% of cases. It is more common in adolescents and adults than younger children, and is associated with increased severity with increasing age. Renal involvement should be monitored with BP and urinalysis, initially at 7 and 14 days after presentation and then monthly until 12 months. Of the other less common complications it is important to know that intussusception is more likely in adolescent age groups and therefore abdo USS is indicated in patients with abdominal pain.

Henoch-Schönlein Purpura Usually self-limiting Supportive treatment with analgesia Corticosteroids if arthralgia and GI symptoms Follow up if arthralgia or renal involvement Excellent long-term prognosis 1/3 of patients experience recurrence HSP is a self-limiting illness with no treatments proven to shorten the duration or reduce the incidence of complications, therefore treatment is usually supportive, with analgesia. NSAIDs can be especially effective but need to be used with caution if there is evidence of renal insufficiency. Corticosteroids may be useful for arthralgias and gastrointestinal dysfunction. There is no consistent evidence for use of immunosuppressive treatments, though azathioprine and cyclophosphamide may be of some benefit. Patients need rheumatology and/or renal follow up if they have arthralgia or renal involvement. Otherwise they can be followed up by their GP with repeat BP measurement and urine dips at 7 and 14 days, and 1, 3, 6 and 12 months. The long-term prognosis is excellent, with the majority of cases resolving spontaneously without treatment. Initial attacks may last up to several months, and one third of patients experience recurrence at some point.

Key Facts HSP follows a viral illness in >50% of cases Adults are at higher risk of renal involvement and more severe renal disease Treatment is supportive with analgesia Steroids may help for arthralgia or GI symptoms Rheumatology F/U if arthralgia or renal involvement Urinalysis for 12 months after the episode

Thanks Lydia Burland