Chapter 138- Cutaneous necrotizing venulitis Flashcards
The signature lesions of cutaneous necrotizing venulitis
Palpable purpura
The most common subtype of CNV
Cutaneous small vessel vasculitis
Most common form of idiopathic CNV in children
IgA vasculitis
Factor significantly associated with shorter survival in CNV
Older than 65 years old
Factors associated with relapse
- Vascular thrombosis was present in skin biopsy
- (+) ANCA
- Hepatic liver enzymes were elevated
- (+) peripheral neuropathy
Most common sites of CNV
Lower legs»_space; back and gluteal regions
Most consistent laboratory abnormality finding
Elevated ESR
Lesions of cutaneous necrotizing venulitis are common on dependent areas such as
Lower extremities»_space; back, gluteal area
Most common precipitating causes of CNV
Infections
Drugs
Histopath criteria of CNV
- Necrosis of blood vessels with deposition of fibrinoid material
- Dermal cellular infiltrates that consist of neutrophils, mononuclear cells, and extravasated erythrocytes
CNV in association with systemic involvement of blood vessels
Hypersensitive Angiitis/Vasculitis
Systemic polyangiitis
Microscopic polyangiitis
Most common etiology of CNV
Idiopathic
CNV is associated with the ff diseases (4)
Rheumatoid arthritis
Sjogren syndrome
SLE
Hypergammaglobulinemic purpura
Dermatomyositis in adults may be associated with vasculitis of GIT
True or False
False, children
Solid malignancies are the most common malignancies associated with CNV.
True or False
False, hematologic malignancies like CML, NHL, HL, CLL, AML, myelodysplastic syndrome
The most common cutaneous feature in patients with ANCA
Palpable purpura
IgA vasculitis occurs in __% of children
75
Preceded by URTI but presents with abdominal pain, melena, arthralgia, hematuria.
Henoch Schonlein purpura/ IgA vasculitis/ anaphylactoid purpura
Predictive factor for long term renal involvement in HSP
Involvement of UE and LE
_% associated with relapse of HSP and involves one of the ff factors.
23%
- Longer duration of initial episode
- Joint manifestations
- Lack of infectious cause
Acute hemorrhagic edema in infancy occurs in less than __ years old, with slight predominance in __.
2; M
Initial sign of AHE
Facial edema
Differentiating factors of IgA vasculitis and AHE
Age distribution
Lack of systemic features
Resolution in 1-3 weeks without sequelae
Edematous form of necrotizing venulitis occurs in patients with serum sickness, CT disorders, hematologic and other malignant conditions, infections, and physical urticarias
Urticarial vasculitis/venulitis
Disease with severe systemic manifestations, hypocomplemetemia with low C1q levels
Hypocomplementemic urticarial vasculitis syndrome
Episodes of urticarial venulitis associated with monoclonal IgMkappa M component, fever, LN, hepatosplenomegaly, bone pain, sensorimotor neuropathy, and renal failure
Schnitzler syndrome
Evolution into hematologic malignancy has been reported in __% of Schnitzler syndrome patients
15
Urticarial vasculitis may persist for up to
3- 5 days
Erythema induratum has been associated with the ff infections
M. Tb
Hep C virus
Nodular vasculitis may present as tender red subQ nodules on the ff areas
Thighs
buttocks
Trunks
Arms
Recurrent painful ulcers of LE associated with persistent livedo reticularis that is deep purple in color
Livedoid vasculopathy
Sclerotic pale areas surrounded by telangiectasias
Atrophie blanche
Systemic involvement is a feature of idiopathic livedoid vasculopathy.
True or False
False, not
Condition in which livedoid vasculopathy and livedo racemosa are associated with ischemic cerebrovascular lesions, hypotension, and extracerebral arterial and venous thromboses
Sneddon syndrome
In livedoid vasculopathy, patients have ___ of the LE
Arteriosclerosis or stasis
Individuals with recurrent pruritic and purpuric papular skin lesions, urticarial plaques and angioedema
Eosinophilic vasculitis
__ genotype is associated in patients with IgA vasculitis
HLADRB1
Increased prevalence of the ff genotypes in CNV patients
HLA A11
HLABw 35
Most consistent abnormal lab finding in patients with CNV
Elevated ESR
Treatment for schnitzler syndrome
IL-1 inhibitors
Anakinra, Canakinumab, Rilonacept
Agents used in the treatment of CNV
H1 antihistamines + NSAIDs
Associated with clearing of HepC infections
IFN alpha
Dominant subclass deposited in IgA vasculitis
IgA1
Extensive areas of purpura with slate gray color
DIC (purpura fulminans)
Finite numbers of hemorrhagic papules, pustules, vesicles that are distributed over acral areas
Septic emboli
The numbers of __ decreases and __ increases at 48 hours
Neutrophils
Monocytes/Macrophages
Present in increased numbers in drug-induced CNV
Eosinophils
Hypocomplementemia occurs in __% of urticarial vasculitis
40
__ induces expression of E selectin on endothelial cells and is chemotactic for leukocytes
CGRP
Necrotizing vasculitis in the skin predominantly involves
Venules
The most common cutaneous feature in patients with ANCA
Palpable purpura
Rheumatoid arthritis + CNV presents with (3)
Peripheral neuropathy
Nailfold infarcts
Digital gangrene
Sjogren syndrome + CNV presents with (4)
Peripheral neuropathy
Articular involvement
Renal involvement
Raynaud phenomenon
The most commonly recognized infectious agents for CNV (5)
B hemolytic streptococcus Staphylococcus aureus Mycobacterium leprae Hepatitis B Hepatitis C
Necrotizing vasculitis due to direct invasion of blood vessel wall is due to (6)
Neisseria meningitidis, Neisseria gonorrhoeae Pseudomonas Haemophilus Rickettsia Candida Infectious endocarditis
The most common drugs incriminated in CNV (9)
B lactams NSAIDs(paracetamol) Allopurinol Anticonvulsant (phenytoin) GM CSF Hydralazine Isotretinoin MTX Penicillamine
