CUTANEOUS VASCULITIS. Katharine Warburton ST6 Dermatology

Transcription

1 CUTANEOUS VASCULITIS Katharine Warburton ST6 Dermatology

2 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

3 CASES

4 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

5 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

6 CASE 3 Female, 54 Acute presentation ascites, leg swelling, vomiting Hep C positivity identified Perforated gastric ulcer, multi organ failure

7 THE THEORY Vasculitis = inflammation of blood vessel

8 CLASSIFYING VASCULITIS Small vessels <50micrometers Medium vessels micrometer Large vessel

9 CATEGORISING CUTANEOUS VASCULITIS Reactive/secondary IgA vasculitis (Henoch-Schonlein Purpura, HSP) Urticarial vasculitis Cryoglobulinaemic ANCA associated Polyarteritis nodosa Primary systemic vasculitidies (<4%) Nodular

10 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

11 CATEGORISING CUTANEOUS VASCULITIS IgA vasculitis (Henoch-Schonlein Purpura, HSP) Children/younger adults Post strep Purpuric rash (to buttocks) Joint and abdominal pain IgA nephropathy

12 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

13 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

14 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% % necrotizing glomerulonephritis % 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

15 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

16 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

17 RELEVANT FINDINGS IN THE SKIN Petichiae, Purpura Palpable Purpura, Haemorrhagic Bullae

18 RELEVANT FINDINGS IN THE SKIN Purpura, Haemorrhagic bullae, ulceration

19 RELEVANT FINDINGS IN THE SKIN

20 RELEVANT FINDINGS IN THE SKIN Vasculitic nodules (can ulcerate) Livedo

21 RELEVANT FINDINGS IN THE SKIN Urticarial vasculitis urticaria and hyperpigmentation

22 RELEVANT FINDINGS IN THE SKIN Resolving vasculitis Red purple brown

23 PITFALLS/MIMICS Capillaritis Leaky vessels (stasis/pressure) Platelet disorders, anticoagulated

24 PITFALLS/MIMICS Vaso-occlusive disorders hypercoaguable states, thrombosis, emboli (eg cholesterol, septic).

25 PITFALLS/MIMICS Scurvy

26 INITIAL MANAGEMENT IN GIM

27 INITIAL MANAGEMENT APPROACH IN GIM 3 key questions: Is there systemic involvement? Is there an obvious cause? How severe is this?

28 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

29 APPROACH: CAUSE? Any new meds? Recent illness? Travel and sexual history Relevant PMH

30 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

31 APPROACH: SEVERITY Skin compromise? Ulceration Digit ischaemia

32 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)

33 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!!

34 MANAGEMENT PRINCIPLES

35 TREATMENT PRINCIPLES If systemic involvement involve relevant specialty Treat a cause Eg infection, withdraw causative drugs etc

36 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)

37 BACK TO THE CASES

38 SUMMARY

39 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?