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 Polyarteritis nodosa Primary systemic vasculitidies (<4%) Nodular
CATEGORISING CUTANEOUS VASCULITIS Reactive/secondary (cutaneous small vessel vasculitis) Mostly limited to skin 7-10 days after exposure to enticing agent Longer (7-21 days) for drugs IgA vasculitis (Henoch-Schonlein Purpura, HSP) Urticarial vasculitis Causes 15% infection 15% drugs 10% inflammatory/connective tissue disorder 5% cancer 50% unknown Exercise induced Cryoglobulinaemic ANCA associated Polyarteritis nodosa 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 IgA polyangiitis vasculitis (Henoch-Schonlein granulomatosis with Purpura, HSP) Urticarial vasculitis Cryoglobulinaemic ANCA associated Polyarteritis nodosa Nodular Eosinophilic polyangitis (syn CSS) Granulomatosis with polyangiitis (syn. Wegner s) panca (MPO) in 60-80% panca (MPO) in 50-80% canca (PR3) in 90% - 70-90% necrotizing glomerulonephritis - 25-50% respiratory complications Constitutional symptoms common for all - Asthma - Eosinophilia - Necrotizing vasculitis with extravascular granulomas 3 stages Skin findings usually palpable purpura. Rarely nodules, ulcers, digital ischaemia, livedo. - 50% - 70% - 40% Triad: - Vasculitis - 80% Necrotizing granulomas respiratory tract - 75% glomerulonephritis
CATEGORISING CUTANEOUS VASCULITIS Polyarteritis nodosa Medium vessel: skin = nodules, ulcers, livedo Cutaneous PAN mostly just skin relapsing remitting constitutional upset Systemic PAN Multiorgan Skin, kidney, GI, neuro, cardiac Chronic, insidious 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 Petichiae, Purpura Palpable 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 > vasculitis screen Viral serology eg EBV 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 Mostly supportive grade 1 compression stockings ibuprofen Leg elevation and rest 90% self resolve Voltaire: The art of medicine consists of amusing the patient while nature cures the disease 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?