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)
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
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?