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Bolognia Book Club > Chapter 25 - Cutaneous Vasculitis > Flashcards

Chapter 25 - Cutaneous Vasculitis Flashcards

1
Q

When referring to “small vessel vasculitis”, what type of vessels could be involved?

A

Arterioles, capillaries, and post-capillary venules which are found in the superficial and mid-dermis of the skin

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2
Q

Increased neutrophil and lymphocyte endothelial cell adhesion results from an increase in which two selectin molecules on the endothelial cells.

A

E-selectin and P-selectin

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3
Q

Increased neutrophil and lymphocyte endothelial cell adhesion results from an increase in which three members of the immunoglobin superfamily on the endothelial cells?

A

ICAM-1, VCAM-1, and PECAM-1

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4
Q

What does “ANCA” stand for?

A

Antineutrophil cytoplasmic antibodies (ANCA)

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5
Q

List the molecules found within the cytoplasm of neutrophils that are potential targets for ANCA antibodies.

A

Proteinase 3 (PR3) and myeloperoxidase (MPO)

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6
Q

Why do lesions of palpable purpura in small vessel vasculitides occur predominantly on dependent sites, as well as under tight-fitting clothing?

A

Because hydrostatic pressure and stasis are involved in the pathophysiology of small vessel vasculitis. The slow flow of blood in these areas allows for deposition of the immunoglobulins and complement

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7
Q

If you suspect a cutaneous vasculitis, should you biopsy a new or old lesion for H&E? What about for DIF?

A

New lesions should be biopsied for both H&E and DIF

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8
Q

In small vessel vasculitis, what will the DIF show?

A

C3, IgG, IgM and/or IgA in a granular pattern within the vessel wall

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9
Q

True or false: when a patient has constitutional (fevers, weight loss, arthralgias) or musculoskeletal symptoms, your diagnosis of small vessel vasculitis of the skin should likely be reassessed.

A

False; patients with small vessel vasculitis of the skin often present with constitutional symptoms and musculoskeletal symptoms

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10
Q

True or false: when a patient has gastrointestinal, genitourinary, or neurologic symptoms, your diagnosis of small vessel vasculitis of the skin should likely be reassessed.

A

True; these symptoms should raise the possibility of a systemic vasculitis

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11
Q

What percentage of patients with small vessel cutaneous vasculitis will have resolution of cutaneous lesions within several weeks?

A

90%; 10% will have chronic or recurrent disease

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12
Q

True or false: small vessel cutaneous vasculitis can occur in the context of an autoimmune connective tissue disease or neoplasm.

A

True; if so, this will affect the patient’s prognosis

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13
Q

List four small vessel cutaneous vasculitides.

A

HSP, acute hemorrhagic edema of infancy, urticarial vasculitis, and erythema elevatum diutinum

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14
Q

List two large vessel vasculitides.

A

Temporal arteritis and Takayasu’s arteritis

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15
Q

List the two classic medium-vessel vasculitides.

A

Classic (systemic) PAN and cutaneous PAN

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16
Q

List the three ANCA-associated small-medium vessel vasculitides.

A

Microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss

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17
Q

True or false: cryoglobulinemia can cause a small-medium vessel vasculitide.

A

True

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18
Q

List four causes of secondary small-medium sized vasculitides.

A

Infections, autoimmune connective tissue diseases, drugs, and neoplasms

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19
Q

Approximately what percentage of cutaneous small vessel vasculitides are idiopathic? What percentage are secondary to another cause (e.g. infection, autoimmune disorder, etc.)?

A

About 50% idiopathic; 50% secondary to another cause

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20
Q

What is the tetrad of clinical findings of HSP?

A

Palpable purpura, arthritis, abdominal pain, and hematuria

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21
Q

What size of vessel is involved in HSP? What type of immunoglobin is involved?

A

Small vessels become coated in IgA

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22
Q

What is the most common form of vasculitis in children?

A

HSP

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23
Q

Does HSP follow a seasonal pattern? If so, when is it most common?

A

Yes; peak incidence during winter

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24
Q

What type of infection typically precedes HSP?

A

Upper respiratory tract infection

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25
Q

Over what general time frame do skin lesions associated with HSP regress?

A

Weeks to months

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26
Q

True or false: children or young adults with a history of HSP have an increased risk of pregnancy-induced hypertension.

A

True

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27
Q

Although the treatment of HSP is usually supportive (because the skin lesions resolve in weeks to months), what drug has been shown to decrease the duration of skin lesions?

A

Systemic steroids and dapsone; systemic steroids also help with the arthritis and abdominal pain

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28
Q

What’s the rare form of cutaneous vasculitis that was once thought to be a benign form of HSP?

A

Acute hemorrhagic edema of infancy; it’s now known to be it’s own entity

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29
Q

What’s the most common eponymn associated with acute hemorrhagic edema of infancy?

A

Finkelstein’s disease

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30
Q

How old are children that are typically affected by Finkelstein’s disease?

A

Children with acute hemorrhagic edema of infancy (Finkelstein’s disease) are usually between 4 - 24 months old (i.e. less than 2 years old)

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31
Q

True or false: patients with acute hemorrhagic disease of infancy (Finkelstein’s disease) typically present with fever and appear toxic.

A

False; patients may present with fever, but otherwise they appear well

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32
Q

True or false: proteinuria often accompanies Finkelstein’s disease (acute hemorrhagic edema of infancy).

A

False; there is no extracutaneous involvement

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33
Q

True or false: acute hemorrhagic edema of infancy (Finkelstein’s disease) often occurs after a recent infection, drug exposure, or immunization.

A

True

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34
Q

What two types of urticarial vasculitis are classically described?

A

Hypocomplementic and normocomplementic

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35
Q

True or false: urticarial vasculitis is often associated with SLE.

A

True

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36
Q

Which type of urticarial vasculitis follows a more benign course? Hypocomplementic or normocomplementic?

A

Normocomplementic; it typically resolves within 3 years

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37
Q

Which patients with urticarial vasculitis, those with the hypo- or normocomplementic type, are more likely to have extra cutaneous involvement?

A

Those with the hypocomplementic form

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38
Q

Urticarial vasculitis associated with a monoclonal IgM gammopathy and two of a list of systemic findings is known as:

A

Schnitzler’s syndrome

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39
Q

True or false: histologically, urticarial vasculitis will show LCV.

A

True

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40
Q

Name the small vessel vasculitis that presents with symmetric red-violet to red-brown papules and plaques on the extensor surfaces. The skin lesions tend to be persistent.

A

Erythema elevatum diutinum

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41
Q

How long does it typically take for erythema elevatum diutinum to resolve?

A

The disease has a chronic course: between 5-10 years, although many cases lasting over 40 years have been described

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42
Q

What is the classic unique histologic feature of erythema elevatum diutinum that is seen in late-stage lesions?

A

Extracellular cholesterol

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43
Q

What drug has been reported to induce a dramatic improvement in erythema elevatum diutinum (although there’s often a relapse when the medication is discontinued)?

A

Dapsone

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44
Q

What are the three types of cryoglobulins?

A

Type I: monoclonal IgM or IgG
Type II: monoclonal IgM against polycloncal IgG
Type III: Polyclonal IgM against IgG

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45
Q

What type of vasculitis is seen with type I cryoglobulinemia?

A

None; vasculitis is only seen with types II and III

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46
Q

What three organ systems are most commonly affected by cryoglobulins?

A

Skin, kidneys, and peripheral nervous system

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47
Q

What two infectious diseases have cryoglobulinemic vasculitis been associated with?

A

HCV and HBV; 70-90% of patients with essential mixed cryoglobulinemia have HCV and 5% have HBV

48
Q

True or false: mixed cryoglobulinemic disease has been associated with HCV, HBV, HIV, auto-immune disease, lymphoproliferative disorders, and certain carcinomas.

A

False; mixed cryoglobulinemic disease has been associated with HCV, HBV, HIV, auto-immune disease, and lymphoproliferative disorders, but not carcinomas.

49
Q

True or false: the three main ANCA-associated vasculitides include Wegener’s, Churg-Strauss, and mixed essential cryoglobulinemia.

A

False; the three main ANCA-associated vasculitides include Wegener’s, Churg-Strauss, and microscopic polyangiits (not mixed essential cryoglobulinemia)

50
Q

What is the target of the C-ANCA antibody? What about the P-ANCA antibody?

A

C-ANCA: proteinase 3 (PR3)

P-ANCA: myeloperoxidase (MPO)

51
Q

What does the “C” and “P” stand for in C-ANCA and P-ANCA?

A

Cytoplasmic-ANCA

Perinuclear-ANCA

52
Q

What type of ANCA is associated with microscopic polyangiitis?

A

Both C-ANCA and P-ANCA are associated with microscopic polyangiitis; 30% of the time with C-ANCA and 60% of the time with P-ANCA

53
Q

What percentage of patients with Wegener’s will have a positive C-ANCA?

A

80%

54
Q

What percentage of patients with Churg-Strauss will have a positive P-ANCA?

A

60%

55
Q

True or false: ANCA-associated vasculitides are classically characterized by a lack of immune complex deposition in the renal vessels (“pauci-immune”; although this may not be true in the skin).

A

True

56
Q

True or false: microscopic polyangiitis is not recognized as an entity in the American College of Rheumatology classification scheme.

A

True; most patients are instead given the diagnosis of PAN or Wegener’s
*Note: microscopic polyangiitis is considered a diagnosis under the Chapel Hill Consensus conference classification scheme

57
Q

Why is microscopic polyangiitis named this way?

A

The name reflects the pathophysiology; it reflects the fact that capillaries, venules, and veins can be involved, in addition to arteries and arterioles (“POLYangiitis”)

58
Q

True or false: like PAN, microscopic polyangiits is associated with HBV infection.

A

False; PAN is associated with HBV but microscopic polyangiits is NOT associated with HBV infection

59
Q

What percentage of patients with microscopic polyangiitis will develop renal disease?

A

90%; classically a pauci-immune crescentic necrotizing glomerulonephritis

60
Q

Will the necrotizing vasculitis seen in microscopic polyangiitis be continuous or discontinuous?

A

It will be discontinuous (segmental)

61
Q

True or false: patients with microscopic polyangiits have a higher rate of relapse than patients with classic PAN.

A

True

62
Q

What is the classic triad associated with Wegener’s granulomatosis?

A

Granulomatous inflammation of the upper and lower airways, systemic necrotizing small vessel vasculitis, and pauci-immune glomerulonephritis

63
Q

True or false: nasal carriage of S. aureus is associated with relapse of Wegener’s granulomatosis.

A

True

64
Q

True or false: ulcers often develop in patients with Wegener’s granulomatosis that are frequently misdiagnosed as pyoderma gangrenosum.

A

True

65
Q

True of false: nodules, especially common on the elbows, can often occur in Wegener’s granulomatosis. However, unlike in rheumatoid nodules, they never ulcerate.

A

False; nodules on the elbows commonly DO occur in Wegener’s granulomatosis, however they COMMONLY ulcerate, while the nodules associated with rheumatoid arthritis virtually never ulcerate

66
Q

What percentage of patients with Wegener’s granulomatosis will have a positive rheumatoid factor?

A

50%

67
Q

What percentage of patients with Wegener’s granulomatosis will have a positive C-ANCA?

A

80%

68
Q

Is the inflammation that’s seen histologically in Wegener’s granulomatosis granulomatous or non-granulomatous.

A

Granulomatous; hence the name Wegener’s granulomatosis

69
Q

True or false: Churg-Strauss has a lower incidence of renal disease than Wegener’s and is usually associated with asthma and eosinophilia.

A

True

70
Q

True or false: histologically, microscopic polyangiitis lacks granulomatous inflammation.

A

True

71
Q

What is the neurologic complication that commonly occurs in patients with Churg-Strauss (70% of patients)?

A

Mononeuritis multiplex

72
Q

True or false: granulomatous inflammation of the myocardium can occur in both Wegener’s and Churg-Strauss.

A

False; it only occurs in Churg-Strauss and it’s the leading cause of death

73
Q

What is the unique hematologic abnormality associated with Churg-Strauss?

A

Eosinophilia

74
Q

What percentage of patients with Churg-Strauss will have a positive P-ANCA?

A

60%

75
Q

True or false: 10-15% of patients with Churg-Strauss will have a positive C-ANCA.

A

True

76
Q

True or false: just like in eosinophilic fasciitis, in a biopsy of a papulonecrotic lesion of Churg-Strauss, the lesion itself will not have many eosinophils (even though there is typically dramatic peripheral eosinophilia).

A

False; in Churg-Strauss, biopsies typically have lots of eosinophils in addition to there being dramatic peripheral eosinophila; this is in contrast to eosinophilic fasciitis (a completely unrelated disorder) in which there is typically peripheral eosinophila, but few eosinophils in the biopsy specimen

77
Q

True or false: asthma and allergic rhinitis are typically associated with Churg-Strauss.

A

True

78
Q

What size of vessel does PAN affect?

A

Medium sized vessels

79
Q

What percentage of cases of PAN are classic (systemic), and what percentage are cutaneous (limited to the skin)?

A

10% cutaneous and 90% classic (systemic)

80
Q

True or false: cutaneous PAN follow a benign course.

A

True; although the course can be very chronic

81
Q

What is the most common infectious trigger of classic (systemic) PAN?

A

HBV

82
Q

What percentage of patients with classic (systemic) PAN have skin findings?

A

50%; the most common findings are livedo reticularis and “punched-out” ulcers

83
Q

True or false: in male patients with classic (systemic) PAN, orchitis frequently occurs.

A

True; extracutaneous disease is the rule in classic (systemic) PAN

84
Q

Which form of PAN is more common in children: classic (systemic) or cutaneous?

A

Cutaneous

85
Q

What infectious disease is cutaneous PAN often associated with in children?

A

Streptococcal infections; in adults the association is with HBV

86
Q

True of false: patients with cutaneous PAN may have systemic symptoms.

A

True, despite the fact that they have “disease limited to the skin”. The systemic symptoms are limited to fever, myalgias, arthralgias, and peripheral neuropathy

87
Q

True or false: the histology of PAN involves segmental necrotizing vasculitis of medium-sized arteries.

A

True

88
Q

True or false: digital necrosis can occur in PAN.

A

True; sometimes this improves with IV prostaglandins or calcium channel blockers

89
Q

True or false: in a patient with a suspected vasculitis and hypertension, a medium vessel vasculitis, involving the intrarenal vessles, may be present.

A

True

90
Q

What size of vessel is typically involved if palpable purpura, urticarial papules, pustules, vesicles, and petechiae are present?

A

Small vessel

91
Q

What type of skin findings are associated with medium vessel vasculitides?

A

Subcutaneous nodules, livedo reticularis, ulcerations, and digital infarcts

92
Q

True or false: biopsies taken from nodules tend to have a higher diagnostic yield for medium sized vessel vasculitides than those taken from an ulcer edge or livedo reticularis.

A

True

93
Q

True or false: if a biopsy of livedo reticularis is planned, one should biopsy in the center of the circular livedo segment, not in the radiating peripheral erythema.

A

True; the peripheral erythema represents venous cyanosis or congestion

94
Q

True or false: ulcers should be biopsied at their edge.

A

True; because incidental vasculitis can often be found underlying ulcers and is non-diagnostic

95
Q

What is the classification for vasculitis of small vessels and its subclassifications?

A

Cutaneous small vessel vasculitis. Henoch Schonlein purpura, acute hemorrhagic edema of infancy, urticarial vasculitis, erythema elevatum diutinum.

96
Q

What are the classifications for vasculitis of small-medium vessels and their subclassifications?

A

Secondary (infection, inflammatory disorders, drug, neoplasm). Cryoglobulinemia. ANCA associated (microscopic polyangiitis, Wegener’s granulomatosis, Churg Strauss syndrome).

97
Q

What is the classfication for vasculitis of medium vessels and its subclassifications?

A

Polyarteritis nodasa/PAN. Classic/systemic PAN, cutaneous PAN.

98
Q

What are the classifications for vasculitis of large vessels?

A

Temporal arteritis, Takayasu’s arteritis.

99
Q

What depositions are most frequently seen on DIF of patient with cutaneous vasculitis?

A

C3, IgG, IgM, IgA

100
Q

What is the classic tetrad of symptoms in HSP?

A

Palpable purpura, arthritis, abdominal pain, hematuria.

101
Q

What increases the risk of long term renal impairment in HSP by 10x?

A

Nephritic or nephrotic syndrome

102
Q

What are some of the differences between HSP and hemorrhagic edema of infancy?

A

AHEI, <2yo, affects only skin, short duration (1-3wks)

103
Q

What other systemic disorders can urticarial vasculitis be associated with?

A

Autoimmune CTD (Sjogren’s syndrome, SLE), infections, drugs, malignancies, serum sickness

104
Q

How does urticarial vasculitis differ from chronic urticaria (ie. both have urticarial lesions lasting >24hrs)?

A

Lesions associated with burning, pain (rather than pruritus), resolve with PIH, truncal and proximal extremities (rather than acral surfaces)

105
Q

What infections are most commonly associated with erythema elevatum diutinum (EED)?

A

B-hemolytic strep, HBV, HIV

106
Q

What is the histology of late lesions in EED?

A

Minimal inflammatory infiltrate, marked perivascular fibrous thickening, vertically oriented capillaries, extracellular cholesterol deposits

107
Q

What infection is most commonly associated with cryoglobulinemic vasculitis?

A

HCV (50% with HCV have CV, but only 5% develop overt CV)

108
Q

What are the 2 clnically relevant patterns of immunofluorescent staining with ANCA?

A
  1. cyptoplasmic ANCA directed against Ag proteinase 3 (c-ANCA, PR3); 2. perinuclear ANCA directed against Ag myeloperoxidase (p-ANCA, MPO)
109
Q

What are some differences between PAN and microscopic polyangiitis?

A

MP has more association with small vessel vasculitic changes (eg. glomerulonephritis, pulmonary hemorrhage), is not associated with HBV infection, role of p-ANCA and is pauci-immune, more likely to relapse. They rarely present with HTN, aneurysms

110
Q

What is the triad of Wegener’s granulomatosis?

A

granulomatous inflammation of resp tract, vasculitis (systemic, necrotizing, small vessel), pauci-immune glomerulonephritis

111
Q

What are some of the symptoms affecting the resp tract in WG?

A

Recurrent epistaxis, mucosal ulcerations, nasal septal performation, saddle nose deformity, dyspnea, cough, hemoptysis, pleuritis

112
Q

What are some of the key features of Churg Strauss syndrome?

A

Asthma, allergic rhinitis, eosinophilia, necrotizing granulomatous vasculitis

113
Q

What are the 3 phases of CSS?

A
  1. allergic rhinitis, nasal polyps, asthma; 2. peripheral eosinophilia, URTI/LRTI, GI symptoms; 3. systemic vasculitis with granulomatous inflammation
114
Q

How is cutaneous PAN different from classic PAN?

A

Skin limited, benign, but chronic, most common in children, 10% of all PAN, associated with strep, parvovirus B19, HIV

115
Q

What is the leading cause of death in CSS?

A

Granulomatous inflammation of the myocardium

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