Chapter 138- Cutaneous necrotizing venulitis Flashcards

1
Q

The signature lesions of cutaneous necrotizing venulitis

A

Palpable purpura

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2
Q

The most common subtype of CNV

A

Cutaneous small vessel vasculitis

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3
Q

Most common form of idiopathic CNV in children

A

IgA vasculitis

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4
Q

Factor significantly associated with shorter survival in CNV

A

Older than 65 years old

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5
Q

Factors associated with relapse

A
  1. Vascular thrombosis was present in skin biopsy
  2. (+) ANCA
  3. Hepatic liver enzymes were elevated
  4. (+) peripheral neuropathy
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6
Q

Most common sites of CNV

A

Lower legs&raquo_space; back and gluteal regions

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7
Q

Most consistent laboratory abnormality finding

A

Elevated ESR

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8
Q

Lesions of cutaneous necrotizing venulitis are common on dependent areas such as

A

Lower extremities&raquo_space; back, gluteal area

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9
Q

Most common precipitating causes of CNV

A

Infections

Drugs

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10
Q

Histopath criteria of CNV

A
  1. Necrosis of blood vessels with deposition of fibrinoid material
  2. Dermal cellular infiltrates that consist of neutrophils, mononuclear cells, and extravasated erythrocytes
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11
Q

CNV in association with systemic involvement of blood vessels

A

Hypersensitive Angiitis/Vasculitis
Systemic polyangiitis
Microscopic polyangiitis

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12
Q

Most common etiology of CNV

A

Idiopathic

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13
Q

CNV is associated with the ff diseases (4)

A

Rheumatoid arthritis
Sjogren syndrome
SLE
Hypergammaglobulinemic purpura

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14
Q

Dermatomyositis in adults may be associated with vasculitis of GIT
True or False

A

False, children

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15
Q

Solid malignancies are the most common malignancies associated with CNV.
True or False

A

False, hematologic malignancies like CML, NHL, HL, CLL, AML, myelodysplastic syndrome

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16
Q

The most common cutaneous feature in patients with ANCA

A

Palpable purpura

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17
Q

IgA vasculitis occurs in __% of children

A

75

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18
Q

Preceded by URTI but presents with abdominal pain, melena, arthralgia, hematuria.

A

Henoch Schonlein purpura/ IgA vasculitis/ anaphylactoid purpura

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19
Q

Predictive factor for long term renal involvement in HSP

A

Involvement of UE and LE

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20
Q

_% associated with relapse of HSP and involves one of the ff factors.

A

23%

  1. Longer duration of initial episode
  2. Joint manifestations
  3. Lack of infectious cause
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21
Q

Acute hemorrhagic edema in infancy occurs in less than __ years old, with slight predominance in __.

A

2; M

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22
Q

Initial sign of AHE

A

Facial edema

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23
Q

Differentiating factors of IgA vasculitis and AHE

A

Age distribution
Lack of systemic features
Resolution in 1-3 weeks without sequelae

24
Q

Edematous form of necrotizing venulitis occurs in patients with serum sickness, CT disorders, hematologic and other malignant conditions, infections, and physical urticarias

A

Urticarial vasculitis/venulitis

25
Q

Disease with severe systemic manifestations, hypocomplemetemia with low C1q levels

A

Hypocomplementemic urticarial vasculitis syndrome

26
Q

Episodes of urticarial venulitis associated with monoclonal IgMkappa M component, fever, LN, hepatosplenomegaly, bone pain, sensorimotor neuropathy, and renal failure

A

Schnitzler syndrome

27
Q

Evolution into hematologic malignancy has been reported in __% of Schnitzler syndrome patients

A

15

28
Q

Urticarial vasculitis may persist for up to

A

3- 5 days

29
Q

Erythema induratum has been associated with the ff infections

A

M. Tb

Hep C virus

30
Q

Nodular vasculitis may present as tender red subQ nodules on the ff areas

A

Thighs
buttocks
Trunks
Arms

31
Q

Recurrent painful ulcers of LE associated with persistent livedo reticularis that is deep purple in color

A

Livedoid vasculopathy

32
Q

Sclerotic pale areas surrounded by telangiectasias

A

Atrophie blanche

33
Q

Systemic involvement is a feature of idiopathic livedoid vasculopathy.
True or False

A

False, not

34
Q

Condition in which livedoid vasculopathy and livedo racemosa are associated with ischemic cerebrovascular lesions, hypotension, and extracerebral arterial and venous thromboses

A

Sneddon syndrome

35
Q

In livedoid vasculopathy, patients have ___ of the LE

A

Arteriosclerosis or stasis

36
Q

Individuals with recurrent pruritic and purpuric papular skin lesions, urticarial plaques and angioedema

A

Eosinophilic vasculitis

37
Q

__ genotype is associated in patients with IgA vasculitis

A

HLADRB1

38
Q

Increased prevalence of the ff genotypes in CNV patients

A

HLA A11

HLABw 35

39
Q

Most consistent abnormal lab finding in patients with CNV

A

Elevated ESR

40
Q

Treatment for schnitzler syndrome

A

IL-1 inhibitors

Anakinra, Canakinumab, Rilonacept

41
Q

Agents used in the treatment of CNV

A

H1 antihistamines + NSAIDs

42
Q

Associated with clearing of HepC infections

A

IFN alpha

43
Q

Dominant subclass deposited in IgA vasculitis

A

IgA1

44
Q

Extensive areas of purpura with slate gray color

A

DIC (purpura fulminans)

45
Q

Finite numbers of hemorrhagic papules, pustules, vesicles that are distributed over acral areas

A

Septic emboli

46
Q

The numbers of __ decreases and __ increases at 48 hours

A

Neutrophils

Monocytes/Macrophages

47
Q

Present in increased numbers in drug-induced CNV

A

Eosinophils

48
Q

Hypocomplementemia occurs in __% of urticarial vasculitis

A

40

49
Q

__ induces expression of E selectin on endothelial cells and is chemotactic for leukocytes

A

CGRP

50
Q

Necrotizing vasculitis in the skin predominantly involves

A

Venules

51
Q

The most common cutaneous feature in patients with ANCA

A

Palpable purpura

52
Q

Rheumatoid arthritis + CNV presents with (3)

A

Peripheral neuropathy
Nailfold infarcts
Digital gangrene

53
Q

Sjogren syndrome + CNV presents with (4)

A

Peripheral neuropathy
Articular involvement
Renal involvement
Raynaud phenomenon

54
Q

The most commonly recognized infectious agents for CNV (5)

A
B hemolytic streptococcus
Staphylococcus aureus
Mycobacterium leprae
Hepatitis B
Hepatitis C
55
Q

Necrotizing vasculitis due to direct invasion of blood vessel wall is due to (6)

A
Neisseria meningitidis, Neisseria gonorrhoeae
Pseudomonas
Haemophilus
Rickettsia
Candida
Infectious endocarditis
56
Q

The most common drugs incriminated in CNV (9)

A
B lactams
NSAIDs(paracetamol)
Allopurinol
Anticonvulsant (phenytoin)
GM CSF
Hydralazine
Isotretinoin
MTX
Penicillamine