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UCD Dermatology - Term 2 > Vasculitis > Flashcards

Vasculitis Flashcards

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Dermatology Board Prep flashcards
1
Q

List the 3 subtypes of vasculitis

A
  • Skin limited
  • Primary cutaneous with secondary systemic
  • Systemic vasculitis with cutaneous involvement
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2
Q

List 2 large vessel vasculitides

A
  • Takayasu arteritis
  • Temporal arteritis (Giant cell)
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3
Q

List 2 medium vessel vasculitides

A
  • Polyarteritis nodosa (PAN)
  • Kawasaki disease
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4
Q

List 3 mixed vessel (small/medium)

A
  • ANCA-associated
    • Microscopic polyangiitis
    • Granulomatosis with polyangiitis (Wegener’s)
    • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
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5
Q

List 3 cutaneous small vessel vasculitides (CSVV)

A
  • Henoch-Schönlein purpura
  • Acute hemorrhagic edema of infancy
  • Urticarial vasculitis
  • Erythema elevatum diutinum
  • Secondary causes
    • Drug exposure
    • Infections
    • Malignancies (most often hematologic)
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6
Q

List 3 immune complex small-vessel vasculitides

A
  • Cryoglobulinemic vasculitis
  • IgA vasculitis (Henoch-Schönlein purpura)
  • Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
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7
Q

What are skin lesions associated with small vessel vasculitis?

A
  • Palpable or macular purpura
  • Petechiae
  • Targetoid lesions
  • Vesicles
  • Pustules
  • Urticarial papules
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8
Q

What is the term for the area circled?

A

Neutrophil dust

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9
Q

In small vessel vaculitis, how long after the triggering event do skin lesions appear?

A

7-10 days

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10
Q

What are skin lesions associated with medium vessel vasculitis

A

Livedo racemosa

Retiform purpura

Ulcers

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11
Q

What is the diagnosis?

What type of vessels are affected?

A

IgA vasculitis (HSP)

small vessel

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12
Q

What type of vessels are involved?

A

small vessel

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13
Q

If you are suspecting cutaneous small vessel vasculitis (CSVV), what test should you request with your biopsy?

A

Direct immunofluorescence

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14
Q

What type of biopsy should you take if you are suspecting medium-vessel vasculitis?

A

Deep incisional biopsy into subcutaneous fat

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15
Q

In vasculitis, where should you take the biopsy from ulcers?

A

the edge, including peripheral rim of inflammation

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16
Q

In cutaneous small vessel vasculitis (CSVV), how often is direct immunofluorescence (DIF) positive?

What is typically positive?

What is the expected result in ANCA-associated vasculitis with DIF?

A

80%

C3, IgM, IgA +/- IgG

Negative

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17
Q

Is vasculitis itchy?

A

No, it shouldn’t be

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18
Q

What is the diagnosis?

A

Calciphylaxis

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19
Q

What is the diagnosis?

A

Erythema nodosum

20
Q

What is the diagnosis?

What are 2 characteristics of this disorder?

What is the treatment?

A

Livedoid vasculopathy

  • Stellate atrophy
  • Exquisite pain
  • Induration

Anticoagulation

21
Q

What areas are favoured with cutaneous small vessel vasculitis?

A
  • Trauma
  • Dependent sites
  • Tight fitting clothing (socks)
22
Q

What are the 5 most common causes (in order) of CSVV?

A
  1. Idiopathic
  2. Infection
  3. Autoimmune connective tissue disease
  4. Drugs
  5. Neoplasm
23
Q

What are the affected systems of CSVV?

What % recover?

If chronic course, what is the time frame?

What is the treatment?

A
  • Renal signs and symptoms
  • Arthralgia and arthritis
  • GI signs and symptoms

90% spontaneously recover

10% chronic course, average 28 months (more if arthralgia, cryoglobulinemia)

  • Supportive
  • Eliminate triggers
  • +/- steroids
  • Colchicine or dapsone for chronic disease (rarely Aza, MTX)
24
Q

What are some unique features of adult HSP?

A

More necrotic lesions

More likely to develop chronic kidney disease (30%)

Can be malignancy driven

25
Q

In HSP, what % are < 10 years old

What is the most common associated infection with HSP?

What is the common distribution of skin lesions?

What % have renal involvement?

How long should the patient be screened for renal involvement

A

90%

Streph throat 50% 1-2 weeks prior

Lower extremities, buttocks, sparing flexure surfaces

50%

For 3 months

26
Q

What are poor prognostic factors for renal involvement in HSP?

A
  • Renal failure at time of onset
  • Nephrotic syndrome
  • Hypertension
  • Decreased factor XIII activity
27
Q

What is the treatment of HSP?

A

Supportive

Colchicine/dapsone can decrease duration of skin lesions

Systemic steroids can help arthritis and abdo pain (if severe)

28
Q

What is this diagnosis?

A

Acute hemorrhagic edema of infancy

29
Q

When does acute hemorrhagic edema of infancy typically occur?

What sex does it tend to present in?

What are common triggers?

What is the distribution and features of skin lesions?

When does it resolve?

What is the treatment?

A

4-24 months

Males (70%)

  • Post-infection
  • Post-vaccine
  • Drug related

Edematous, red plaques to face + extremities (non-pitting acral)

1-3 weeks

Treat any underlying infection

30
Q

What is the diagnosis?

A

Urticarial vasculitis

31
Q

What are the common features of urticarial vasculitis

What treatments are recommended?

A
  • Recurrent painful urticarial lesions
  • Last >24 hours
  • Resolve with hyperpigmentation/bruising
  • +/- angioedema
  • Antihistamines
  • +/- oral steroids
  • Colchicine
  • Hydroxychloroquine
  • MMF (mycophenolate mofetil)
32
Q

What is the diagnostic criteria for Hypogomplementemic urticarial vasculitis syndrome (HUVS)?

A

2 MAJOR + ≥2 MINOR Criteria

MAJOR

  • Urticaria > 6 months
  • Hypocomplementemia

MINOR

  • Vasculitis on skin biopsy (leukoclastic vasculitis)
  • Arthralgia or arthritis
  • Uveitis or episcleritis
  • Glomerulonephritis
  • Recurrent abdominal pain
  • Positive C1q precipitin test with low C1q level
33
Q

What is the diagnosis?

A

Erythema elevatum diutinum

34
Q

What are the skin characteristics of erythema elevatum diutinum?

What laboratory investigations would you order?

What is the treatment?

What are associated features of EED?

A
  • Extensor surface involvement
  • Burning
  • Initially erythematous and soft, then become red-brown or violaceous in colour
  • Doughy or firm to palpation (fibrosis)
  • HIV antigen testing
  • SPEP (serum protein electrophoresis)
  • autoimmune disorders (ANA, ESR, CRP, ENA)
  • Diutinum = long lasting
  • Ocular diseases
    • Peripheral keratitis
    • Nodular scleritis
    • Panuveitis
    • Blindness
  • Arthralgia may develop
  • Dapsone
  • Intralesional steroid
35
Q

Which cryoglobulins….

Cause vascular occlusion?

Cause vasculitis?

Are monoclonal?

Are polyclonal?

A

Subtype I

Subtypes II and III

Subtypes I (IgM > IgG) and II (IgM against polyclonal IgG)

Subtype III (IgM against polyclonal IgG)

36
Q

What are associations and clinical manifestations seen with Cryglobulin subtype I?

A

Associations

  • Plasma cell dyscrasias
  • Lymphoproliferative disorders

Clinical manifestations

  • Raynaud’s phenomenon
  • Retiform purpura
  • Gangrene
  • Acrocyanosis
37
Q

What are associations and clinical manifestations seen with Cryglobulin subtypes II and III?

A

Associations

  • Hepatitis C
  • HIV
  • Autoimmune connective tissue diseases
  • Lymphoproliferative disorders

Clinical Manifestations

  • Palpable purpura
  • Arthralgias
  • Glomerulonephritis
  • Peripheral neuropathy
38
Q

What ANCA antigen is associated with…

p-ANCA?

c-ANCA?

A

p-ANCA = perinuclear ANCA = MPO antigen

c-ANCA = cytoplasmic ANCA = PR3 antigen

39
Q

What is the triad associated with granulomatosis with polyangiitis? (Wegener’s)

What type of ANCA is implicated?

What bacteria is associated with relapse of granulomatosis with polyangiitis?

What treatments are advised?

A
  1. Necrotizing granulomatous inflammation of URT/LRT
  2. Pauci-immune glomerulonephritis
  3. Necrotizing small/medium vessel vasculitis

c-ANCA

S. aureus

  • TMP/SMX
  • Steroids
  • MTX
  • Cyclophosphamide/rituximab if severe
40
Q

What is the diagnosis?

A

Microscopic polyangiitis

41
Q

What are characteristics of microscopic polyangiitis (MPA)?

What antigens are seen with this presentation?

What are clinical features of MPA?

A
  • Palpable purpura
  • Splinter hemorrhages
  • Ulcers
  • No granulomas
  • No eosinophilia

60% p-ANCA (anti-MPO), 30% c-ANCA (anti-PR3)

  • Constitutional symptoms (fever, weight loss, arthralgia, myalgia)
  • Renal involvement (>90%)
    • Pauci-immune crescenteric necrotizing glomerulonephritis
  • Pulmonary capillaritis
    • Dyspnea with pulmonary infiltrates
    • Diffuse alveolar hemorrhage
  • Neurologic involvement
    • Peripheral neuropathy
    • Mononeuritis multiplex
42
Q

What is the diagnosis?

A

Eosinophilic granulomatosis with polyangiitis (Churg Strauss)

43
Q

What are the 3 phases of eosinophilic granulomatosis with polyangiitis (Churg Strauss)

What systems are at increased risk?

What antigen is typically implicated?

A

PHASE 1

  • Allergic rhinitis
  • Nasal polyps
  • Asthma x years

PHASE 2

  • Peripheral eosinophilia
  • Respiratory tract infections
  • GI symptoms

PHASE 3 (years to decades after original symptoms)

  • Systemic necrotizing vasculitis with granulomatous inflammation

Cardiac and Neurological

p-ANCA

44
Q

What is the diagnosis if present on back or buttocks?

Where would you biopsy?

A

Livedo racemosa

The pale areas

45
Q

What renal manifestation is associated with polyarteritis nodosa (PAN)?

What diseases are associated with PAN?

What size vessels are involved?

A

Renovascular hypertension

  • Inflammatory bowel disease
  • Leukeomia
  • Hepatitis B + C

Medium

46
Q

What is the diagnosis?

A

Temporal arteritis

UCD Dermatology - Term 2 (14 decks)

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