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CUTANEOUS VASCULITIS Katharine Warburton ST6 Dermatology AIMS

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CUTANEOUS VASCULITIS Katharine Warburton ST6 Dermatology AIMS CUTANEOUS VASCULITIS Katharine Warburton ST6 Dermatology AIMS Clinical cases introduction ‘The theory’ Categorising cutaneous vasculitis Features presenting in the skin Mimics/pitfalls How to initially manage and investigate in GIM When to involve dermatology When is biopsy required Management principles Back to cases CASES CASE 1 57 year old female Mild chronic ITP, untreated (plts 80) Obesity Had 3/7 trimethoprim from GP for uncomplicated UTI Completed 12 days ago Presents with new rash after travelling up from Cornwall Well Obs normal Urine dip normal Initial bloods ok CASE 2 22 year old student Rapidly extending rash 1 wk GP gave antibiotics for folliculitis but progressed Feels ‘not right’, bit achey. Crampy abdo pain. BP 132/75 Urine dip trace of protein Initial bloods ok, vasculitis screen pending CASE 3 Female, 54 Acute presentation ascites, leg swelling, vomiting Hep C positivity identified Perforated gastric ulcer, multi organ failure ‘THE THEORY’ Vasculitis = inflammation of blood vessel CLASSIFYING VASCULITIS Small vessels <50micrometers Medium vessels 50-150 micrometer Large vessel CATEGORISING CUTANEOUS VASCULITIS Reactive/secondary IgA vasculitis (Henoch-Schonlein Purpura, HSP) Urticarial vasculitis Cryoglobulinaemic ANCA associated Primary systemic vasculitidies (<4%) Polyarteritis nodosa Nodular CATEGORISING CUTANEOUS VASCULITIS Reactive/secondary (cutaneous small vessel vasculitis) • IgAMostly vasculitis limited (Henoch to skin-Schonlein Purpura, HSP) • 7-10 days after exposure to enticing agent • UrticarialLonger (7 vasculitis-21 days) for drugs • CryoglobulinaemicCauses 15% infection ANCA15% associated drugs 10% inflammatory/connective tissue disorder 5% cancer Polyarteritis nodosa 50% unknown Exercise induced Nodular CATEGORISING CUTANEOUS VASCULITIS IgA vasculitis (Henoch-Schonlein Purpura, HSP) • Children/younger adults • Post strep • Purpuric rash (to buttocks) • Joint and abdominal pain • IgA nephropathy CATEGORISING CUTANEOUS VASCULITIS Urticarial vasculitis • Nettle rash / hives / urticaria, lesions last longer than 24hours • Leaves brown pigment • 50% unknown cause • SLE, malignancy, viral infections, medications • Complement levels helpful for catergorising • Manage in OP dermatology, unless concerning features CATEGORISING CUTANEOUS VASCULITIS Cryoglobulinaemic • Immunoglobulins soluble in blood but precipitate at lower temperatures – skin, kidneys, nervous system • Types I - Heam malignacies II – lymphoproliferative disorders III – (and II) – Rheumatoid disease, infections • Hep C infection 90% • Testing: immunology Lab LGI • Beware digital ischaemia • Treat underlying disease CATEGORISING CUTANEOUS VASCULITIS Reactive/secondary Microscopic Eosinophilic Granulomatosis with IgApolyangiitis vasculitis (Henochgranulomatosis-SchonleinwithPurpura, polyangiitis HSP) polyangitis (syn CSS) (syn. Wegner’s) pANCA (MPO) in 60-80% pANCA (MPO) in 50-80% cANCA (PR3) in 90% Urticarial vasculitis Constitutional symptoms common for all - 70-90% necrotizing - Asthma Triad: glomerulonephritis Cryoglobulinaemic- Eosinophilia - Vasculitis - Necrotizing vasculitis - 80% Necrotizing - 25-50% respiratory with extravascular granulomas respiratory complications ANCA associated granulomas tract - 75% glomerulonephritis ‘3 stages’ Polyarteritis nodosa Skin findings usually palpable purpura. Rarely nodules, ulcers, digital ischaemia, livedo. - 50% Nodular - 70% - 40% CATEGORISING CUTANEOUS VASCULITIS Polyarteritis nodosa • Medium vessel: skin = nodules, ulcers, livedo Cutaneous PAN Systemic PAN mostly just skin Multiorgan Skin, kidney, GI, neuro, cardiac relapsing remitting Chronic, insidious constitutional upset raised ESR, hypertension ACR 1990 Classification May or may not involve skin • Hep B link (30% cases) • Deep biopsy helpful • Aggressive treatmnet – cyclophosphamide, IVIg CATEGORISING CUTANEOUS VASCULITIS Nodular • Medium vessels – nodules, ulcers, oedema • Usually lower legs (calves), also thighs, arms • ?result of slowed circulation • Casues panniulitis ie inflammation of the fat • Can be a sign of TB RELEVANT FINDINGS IN THE SKIN Palpable Purpura, Petichiae, Purpura Haemorrhagic Bullae RELEVANT FINDINGS IN THE SKIN Purpura, Haemorrhagic bullae, ulceration RELEVANT FINDINGS IN THE SKIN RELEVANT FINDINGS IN THE SKIN Vasculitic nodules (can ulcerate) Livedo RELEVANT FINDINGS IN THE SKIN Urticarial vasculitis – urticaria and hyperpigmentation RELEVANT FINDINGS IN THE SKIN Resolving vasculitis Red purple brown PITFALLS/MIMICS Capillaritis Leaky vessels (stasis/pressure) Platelet disorders, anticoagulated PITFALLS/MIMICS Vaso-occlusive disorders hypercoaguable states, thrombosis, emboli (eg cholesterol, septic). PITFALLS/MIMICS Scurvy INITIAL MANAGEMENT IN GIM INITIAL MANAGEMENT APPROACH IN GIM 3 key questions: Is there systemic involvement? Is there an obvious cause? How severe is this? APPROACH: SYSTEMIC INVOLVEMENT? THINK: renal, GI, lung, neuro Thorough review of systems and full examination Lymphadenopathy Fever, BP Urine dip and PCR FBC, U&E, LFT, ESR, CRP Radiology eg CXR APPROACH: CAUSE? Any new meds? Recent illness? Travel and sexual history Relevant PMH APPROACH: CAUSE? ANCA ANA c3/4 (if low complement consider cryoglobulins, anti-cQ1, anti APS) RhF Immunoglobulins TOP TIP!! Serum electrophoresis, BJP ICE > Adult services A-I ASOT > Dermatology’ Viral serology eg EBV > ‘vasculitis screen Hep B/C, HIV Consider skin biopsy, malignancy screen APPROACH: SEVERITY Skin compromise? Ulceration Digit ischaemia Worried - Unwell patient - Systemic features - Odd sites (not just acral) - Nodular/ ulcerating/ blistering - Ischaemia of extremities Not too worried - Well patient - Screening normal - Obvious trigger - Flat rash, starting to resolve (turning brown) DERMATOLOGY INPUT Threat to tissue/severe Needs active treatment Biopsy requirements less so if uncomplicated cutaenous vasculitis with obvious cause Helpful and accessible if thinking systemic vasculitis, vaso-occlusive Pictures and full history please!! MANAGEMENT PRINCIPLES TREATMENT PRINCIPLES If systemic involvement – involve relevant specialty Treat a cause Eg infection, withdraw causative drugs etc IF SKIN INVOLVEMENT ONLY Mild Voltaire: ‘The art of Mostly supportive medicine consists of grade 1 compression stockings amusing the patient ibuprofen Leg elevation and rest while nature cures the disease’ 90% self resolve Ensure urine dips weekly one month minimum Mod/severe Control (pred, IVMP, AZA, CyA, MTX) Maintain remission (MTX, AZA, MMF, pred, rituximab) BACK TO THE CASES… SUMMARY IN SUMMARY Don’t panic, often well patients don’t need admission or instant dermatology input Exclude systemic involvemnt first Think of causes Assess severity Pictures extremely helpful for discussions Thank you – any questions?.
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