cutaneous vasculitis

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approach to management of cutaneous vasculitis

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  • Approach to the

    management of Cutaneous

    Vasculitis Dr Deepshikha Khanna

    Senior Resident

    UCMS & GTB Hospital,

    Delhi

  • What is vasculitis?

    Inflammation of vessel wall

    Presents as heterogenous mix of clinical syndromes

    Clinical picture depends on

    Size and extent of vessel wall

    Organ system involved

    Varied and overlapping clinical features

    Difficulty in obtaining tissues

    Difficult to diagnose and classify

  • Classification

    Size of vessel wall small/medium/large

    American College of Rheumatology Criteria

    Clinical, historical & histological divisions

    Chappell Hill Conference (CHC) histopathological findings

    Etiological classification primary / secondary

    Cutaneous only / cutaneous with systemic involvement

  • Primary cutaneous vasculitis

  • Etiology

    Idiopathic (45-55%)

    Infection (15-20%)

    Drugs, vaccines,

    chemicals, allergies (10-

    15%

    Neoplasm (5%)

    Connective tissue & other inflammatory diseases (15-

    20%)

  • When to suspect?

    Dependent palpable purpura

    Livido reticularis

    Punched out ulcers

    Subcutaneous nodules

    Bullae, necrosis & ulceration

    Over dependent / pressure areas / trauma prone sites

    Single / recurrent crops, resolving over 3-4 wks

    Leaving ecchymotic stains / hyperpigmentation

  • In the absence of cutaneous findings, suspect if:

    Ischemic S/S

    Multi-system inflammatory disease

    Fever & constitutional symptoms

  • Look for systemic involvement Why?

    May suggest exact type of vasculitis

    Will guide diagnostic approach

    Determine treatment protocol

    Prognosis and follow-up

  • Ask for

    Duration of complaints

    drugs / exposure to chemicals /

    food allergies/travel

    H/o CTD / malignancy / sepsis

    Constitutional symptoms

    Hemoptysis, dyspnoea, cough, wheezing

    Eye / ear symptoms, sinusitis

    Numbness and paresthesias

    Abdominal colic, malena, hematuria,liver dysfunction

  • Look for

    Cutaneous findings

    Morphological type

    Size of vessel involved

    BP, peripheral pulses

    Neurological examination

    Central / peripheral

    Diffuse / focal

    Pleural effusion / pleuritis

    Pericardial effusion

    Musculoskeletal system

  • Vessel sizes and clinical presentation

    Palpable purpura

    Splinter Hmg

    Urticaria

    Vesicles

    Ulcers

    Digital infarcts

    Nodules

    Livedo

    Necrosis

    Gangrene in an extremity

    Hypertension

    Aneurysm

    Dissection

  • CUTANEOUS

    VASCULITIS

    Cryoglobulinemic

    vasculitis

    ANCA associated

    vascilitidis

    Kawasaki disease Polyarteritis nodosa

    Urticarial vasculitis

    Henoch Schonlein

    purpura

    Pulm renal syndrome

    Maculopapular rash

    Periungual & perineal desquamation

    Raynauds phenomenon

    Acrocyanosis

    by cold

    Starbust livedo reticularis

    S/C nodules

    Digital gangrene

    Purpura, colic, diarrhoea, nephritis

    Annular urticarial plaques

    HUVS/SLE,NUV

  • Primary cutaneous small vessel vasculitis

    Idiopathic cutaneous vascilitis

    Without significant extracutaneous / systemic

    involvement

    May also include

    Normocomplementemic urticarial vasculitis

    Essential mixed cryoglobulinemia

    Acute hemorrhagic edema of infancy

  • Mimics of cutaneous vasculitis

    Vascular disorders

    PLC

    Perniosis

    Vascular occlusive disease

    DIC

    Thrombocytopenia

    TTP

    Homocystinuria

    Embolic states

    Sneddons syndrome Cholesterol embolism

    Purpura

    Platelet deficiency

    Drug-induced

    Dermatoses

    Schambergs disease Miscellaneous

    Insect-bite reaction

    Cutaneous lymphoma

  • Mimics of systemic vasculitis

    Antiphospholipid antibody syndrome

    Drug-induced

    Cocaine, amphetamines, ergot-derivatives

    Infection

    SABE

    Embolism

    Cardiac myxoma

    Malignancy

  • Test for

    Initial screening CBC with DLC ESR Urine analysis Stool for occult blood Creatinine Liver function tests CXR Infection screen Hepatitis B and C Cutaneous Bx - H&E & DIF

  • Proceed further based on

    Available clinical clues / lab findings

    Caliber of involved vessels

    Severity of disease process

  • Serologocial ANA ANCA Anti-ds DNA RF

    Histopathology/DIF

    Kidney

    bone marrow

    lung

    Serum-Cryoglobulins Complement (CH50, C3, C4) Protein electrophoresis Immunofixation

    GI evaluation /stool occult blood Nerve conduction study HIV USG Angiography Ophthalmological evaluation

    Additional evaluations

  • Histopathological confirmation required in most cases

    cannot stand by itself

    considerable overlap in pathological patterns

    not diagnostic for specific syndromes

    Can be focal / segmental

    Not all vessels may be involved

    Ideal time -18-36 hrs of age of lesion

    Adequate depth including subcutis if MVV

    Non-ulcerated site / nodule / white centre of a livido segment / active border of ulcer

    Most proximal part of the limb

    Histopathology

  • Histopathological subtypes

    Polymorphonuclear-LCV

    Lymphocytic- EM, EN (late stages)

    Granulomatous-WG, infective, EI, nodular vasculitis

    Eosinophilic-CSS, drug eruptions

    Hyalanizing vasculitis- atrophie blanche

  • LCV defined histopathologically by:

    Polymorphonuclear neutrophil infiltrate

    Affecting superficial post-capillary venules

    Fibrinoid deposits in and around vessel wall

    Endothelial swelling

    Extravasation of RBCs

    Karyorrhexis or leukocytoclasia

  • Fresh non-infarcted most proximal lesion preferably non-lesional skin

    IgA most frequent

    Presence of IgA associated with renal involvement but not with its severity

    Presence of IgG/IgM /absence of IgA related to presence of cryoglobulins

    (Barnadas MA, et al. Int J Dermatol 2004; 43: 19-26)

    Direct immunofluorescence

  • Indications Interpretation

    Cryoglobulins Cryoglobulinemic vasculitis Abnormal LFT High RF

    high clinical suspicion- 3 ve results during active flare up False +ve True +ve indicative of Hep C infec 90% T-II & 70% T-III

    Complement Urticarial vasculitis S/S of SLE

    Cryoglobulinemic vasculitis CTD-SLE/RV/SS Anti C1q precipitins HUV

    ANA S/S of CTD Systemic findings Medium vs wall

    UV(low titre 30-50%) CV (20%)

  • When to order?

    Pulmonary hemorrhage

    Recurrent / long standing - sinusitis / otitis

    Orbital mass

    Glomerulonephritis

    Granulomatous vasculitis

    Interpretation

    Indicate pauci-immune vasculitidis DIF -ve

    Antineutrophilic cytoplasmic antibody (ANCA)

  • Seen in
  • Comparison of the ANCA-associated vasculitidis ANCA WG

    C-ANCA 75-80% P-ANCA 10-15%

    Microscopic polyangiitis

    C-ANCA 25-35% P-ANCA 50-60%

    Churg-Strauss syndrome

    C-ANCA 10-15% P-ANCA 55-60%

    Necroptizing granulomas

    + - +

    Asthma / eosinophilia

    - - +

    Pulmonary +++ ++ +++

    Renal +++ +++ ++

    Cutaneous ++ ++ ++

    ENT +++ + ++

    Musculoskeletal ++ ++ ++

    Neurologic ++ + +++

    Gastrointestinal ++ ++ ++

  • Disease C-ANCA (%) P-ANCA (%)

    Wegeners granulomatosis 75-80 10-15

    Microscopic PAN 25-35 50-60

    Churg-Strauss syndrome 10-15 55-60

  • Vasculitis Therapy Literature - Problems

    1. Numbers small

    2. RCT ???

    3. Blinding?

    4. How to eliminate spontaneous resolution?

    5. Different etiologies, associations & systemic

    involvement

    6. Where are the follow up studies?

    7. Why is there such under-reporting?

  • Exclusion / treatment of systemic disorders

    Remove / treat the cause if any

    In the absence of systemic involvement, even cutaneous LCV of

    long duration not life threatening

    management strategies effective but with limited S/E

    Aim of therapy

    Comfort the patient / reduce symptoms

    Prevent extensive cutaneous infarction & systemic

    complications

    Management

  • PCSVV - often single, episodic, self limited disease

    1st line treatment Conservative therapy

  • Conservative therapy

    Bed rest, elevation of lower extremities

    Warming

    Compression hosiery

    NSAIDs

    Anti-histamines

    Avoid tight clothing / trauma

    Elimination diet

    Antibiotics suspected infection induced

    (none of the conservative measures significantly modify the disease course or prevent recurrences)

    Callen JP. South Med J 1987; 80: 848-51.

  • Five pts with hypersensitivity vasculitis and h/o allergy elimination diet-complete remission in 4, partial in 1-challenge tests-relapse on re-introducing1

    Crossover randomised trial of low antigen diet in 24 pts with mixed cryoglobulinemia 2

    So Search for a history of allergy If positive challenge tests Elimination diets for positive tests not routinely recommended as dual occurrence of food intolerance and allergic vasculitis extremely r

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