Chapter 138- Cutaneous necrotizing venulitis

Question 1

The signature lesions of cutaneous necrotizing venulitis

Answer 1

Palpable purpura

Question 2

The most common subtype of CNV

Answer 2

Cutaneous small vessel vasculitis

Question 3

Most common form of idiopathic CNV in children

Answer 3

IgA vasculitis

Question 4

Factor significantly associated with shorter survival in CNV

Answer 4

Older than 65 years old

Question 5

Factors associated with relapse

Answer 5

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

Question 6

Most common sites of CNV

Answer 6

Lower legs&raquo_space; back and gluteal regions

Question 7

Most consistent laboratory abnormality finding

Answer 7

Elevated ESR

Question 8

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

Answer 8

Lower extremities&raquo_space; back, gluteal area

Question 9

Most common precipitating causes of CNV

Answer 9

Infections

Drugs

Question 10

Histopath criteria of CNV

Answer 10

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

Question 11

CNV in association with systemic involvement of blood vessels

Answer 11

Hypersensitive Angiitis/Vasculitis
Systemic polyangiitis
Microscopic polyangiitis

Question 12

Most common etiology of CNV

Answer 12

Idiopathic

Question 13

CNV is associated with the ff diseases (4)

Answer 13

Rheumatoid arthritis
Sjogren syndrome
SLE
Hypergammaglobulinemic purpura

Question 14

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

Answer 14

False, children

Question 15

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

Answer 15

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

Question 16

The most common cutaneous feature in patients with ANCA

Answer 16

Palpable purpura

Question 17

IgA vasculitis occurs in __% of children

Answer 17

75

Question 18

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

Answer 18

Henoch Schonlein purpura/ IgA vasculitis/ anaphylactoid purpura

Question 19

Predictive factor for long term renal involvement in HSP

Answer 19

Involvement of UE and LE

Question 20

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

Answer 20

23%

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

Question 21

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

Answer 21

2; M

Question 22

Initial sign of AHE

Answer 22

Facial edema

Question 23

Differentiating factors of IgA vasculitis and AHE

Answer 23

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

Question 24

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

Answer 24

Urticarial vasculitis/venulitis

Question 25

Disease with severe systemic manifestations, hypocomplemetemia with low C1q levels

Answer 25

Hypocomplementemic urticarial vasculitis syndrome

Question 26

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

Answer 26

Schnitzler syndrome

Question 27

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

Answer 27

15

Question 28

Urticarial vasculitis may persist for up to

Answer 28

3- 5 days

Question 29

Erythema induratum has been associated with the ff infections

Answer 29

M. Tb | Hep C virus

Question 30

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

Answer 30

Thighs buttocks Trunks Arms

Question 31

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

Answer 31

Livedoid vasculopathy

Question 32

Sclerotic pale areas surrounded by telangiectasias

Answer 32

Atrophie blanche

Question 33

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

Answer 33

False, not

Question 34

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

Answer 34

Sneddon syndrome

Question 35

In livedoid vasculopathy, patients have ___ of the LE

Answer 35

Arteriosclerosis or stasis

Question 36

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

Answer 36

Eosinophilic vasculitis

Question 37

__ genotype is associated in patients with IgA vasculitis

Answer 37

HLADRB1

Question 38

Increased prevalence of the ff genotypes in CNV patients

Answer 38

HLA A11 | HLABw 35

Question 39

Most consistent abnormal lab finding in patients with CNV

Answer 39

Elevated ESR

Question 40

Treatment for schnitzler syndrome

Answer 40

IL-1 inhibitors | Anakinra, Canakinumab, Rilonacept

Question 41

Agents used in the treatment of CNV

Answer 41

H1 antihistamines + NSAIDs

Question 42

Associated with clearing of HepC infections

Answer 42

IFN alpha

Question 43

Dominant subclass deposited in IgA vasculitis

Answer 43

IgA1

Question 44

Extensive areas of purpura with slate gray color

Answer 44

DIC (purpura fulminans)

Question 45

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

Answer 45

Septic emboli

Question 46

The numbers of __ decreases and __ increases at 48 hours

Answer 46

Neutrophils | Monocytes/Macrophages

Question 47

Present in increased numbers in drug-induced CNV

Answer 47

Eosinophils

Question 48

Hypocomplementemia occurs in __% of urticarial vasculitis

Answer 48

40

Question 49

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

Answer 49

CGRP

Question 50

Necrotizing vasculitis in the skin predominantly involves

Answer 50

Venules

Question 51

The most common cutaneous feature in patients with ANCA

Answer 51

Palpable purpura

Question 52

Rheumatoid arthritis + CNV presents with (3)

Answer 52

Peripheral neuropathy Nailfold infarcts Digital gangrene

Question 53

Sjogren syndrome + CNV presents with (4)

Answer 53

Peripheral neuropathy Articular involvement Renal involvement Raynaud phenomenon

Question 54

The most commonly recognized infectious agents for CNV (5)

Answer 54

``` B hemolytic streptococcus Staphylococcus aureus Mycobacterium leprae Hepatitis B Hepatitis C ```

Question 55

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

Answer 55

``` Neisseria meningitidis, Neisseria gonorrhoeae Pseudomonas Haemophilus Rickettsia Candida Infectious endocarditis ```

Question 56

The most common drugs incriminated in CNV (9)

Answer 56

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