22 - 138 - CUTANEOUS NECROTIZING VASCULITIS Flashcards
True about the clinical features on cutaneous necrotizing venulitis except?
a. The eruption most often appears on the lower extremities or over dependent areas, such as the back and gluteal regions.
b. Lesions are also common on the face, palms,
soles, and mucous membranes.
c. Palpable purpura persist for 1 to 4 weeks and resolve.
d. Lesional symptoms include pruritus or burning and, less commonly, pain.
B
Page 2528. The lesions may occur anywhere on the skin but are uncommon on the face, palms, soles, and mucous membranes.
Acute hemorrhagic edema of infancy is distinguished from IgA vasculitis by the following, except:
a. Its age distribution
b. Lack of systemic features
c. Resolution within 1 to 3 weeks without sequelae
d. Often are preceded by a history of a recent upper respiratory tract infection
D
Page 2531. Acute hemorrhagic edema of infancy is distinguished from IgA vasculitis by its age distribution, lack of systemic features, and resolution within 1 to 3 weeks without sequelae.
Most cases of cutaneous necrotizing venulitis are:
a. Associated with connective tissue disease
b. Caused by drugs
c. Idiopathic
d. Triggered by infections
C
Which Ig deposit in venules is associated with absence of autoimmune and inflammatory disorder?
a. IgA
b. IgE
c. IgM
d. IgG
A
Page 2536. IgA deposits were related to absence of autoimmune and inflammatory disorders, IgM deposits to the presence of autoimmune and inflammatory disorders, and IgG deposits to a positive ANCA.
True of urticarial venulitis except
a. The eruption does not exceed 3-5days
b. It is more common in women
c. The lesions are pruritic or possess a burning or painful quality; they usually resolve without residua
d. General features include fever, malaise, and myalgia
A
Page 2531. Although the individual urticarial lesions may last for fewer than 24 hours, they often persist for up to 3 to 5 days.
signature lesions of cutaneous necrotizing venulitis (CNV)
palpable purpura
erythematous to violaceous papules that do not blanch when the skin is pressed
most common precipitating causes of CNV
infections and drugs
The most widely recognized subgroup of idiopathic cutaneous necrotizing vasculitis in children
immunoglobulin A vasculitis
Necrotizing vasculitis in the skin predominantly involves what blood vessels?
venules
AKA cutaneous necrotizing venulitis/vasculitis (CNV), cutaneous small-vessel vasculitis, and leukocytoclastic vasculitis
Associated Chronic Disorders in CNV
■ Rheumatoid arthritis
■ Sjögren syndrome
■ Systemic lupus erythematosus
■ Hypergammaglobulinemic purpura
■ Paraneoplastic vasculitis
■ Cryoglobulinemia
■ Ulcerative colitis
■ Cystic fibrosis
■ Antineutrophil cytoplasmic or antiphospholipid antibody syndromes
possible precipitating events of CNV
■ Bacterial, viral, mycobacterial, and rickettsial infections
■ Therapeutic and diagnostic agents
most common etiology of CNV
idiopathic
the only factor that was significantly associated with a shorter survival in patients with CNV
Age older than 65 years at the outset of vasculitis
areas of predilection of CNV
- lower extremities
- back
- gluteal region
The lesions may occur anywhere on the skin but are uncommon on the face, palms, soles, and mucous membranes
in patients with SLE, the presence of what antibody have a greater risk for the development of CNV?
anti-Ro antibody
most common cutaneous feature in patients with ANCAs
palpable purpura
The most commonly recognized infectious agents to precipitate episodes of CNV
- β-hemolytic Streptococcus,
- Staphylococcus aureus,
- Mycobacterium leprae,
- hepatitides B and C viruses
These medications may cause vasculitis in association with ANCAs
Propylthiouracil and hydralazine
IgA vasculitis (formerly Henoch-Schönlein purpura)
Acute hemorrhagic edema of infancy
Acute hemorrhagic edema of infancy is distinguished from IgA vasculitis by its age distribution, lack of systemic features, and resolution within 1 to 3 weeks without sequelae.
Urticarial venulitis (urticarial vasculitis)
Erythema elevatum diutinum
Nodular vasculitis (erythema induratum)
Livedoid vasculopathy (livedoid vasculitis, livedo vasculitis, segmental hyalinizing vasculitis, atrophie blanche)
Sneddon syndrome
Eosinophilic vasculitis
Erythema induratum has been associated with what infections?
Mycobacterium tuberculosis and hepatitis C virus infection
major pathobiologic mechanism in the production of CNV
immune complexes
most consistent abnormal laboratory finding in patients with CNV
elevated erythrocyte sedimentation rate
histopathologic criteria requisite for the diagnosis of CNV
- **necrosis of the blood vessels with deposition of fibrinoid material **and
- dermal cellular infiltrates that consist of neutrophils with nuclear debris, mononuclear cells, and extravasated erythrocytes
dominant Ig subclass that is deposited in IgA vasculitis as seen in DIF
IgA 1
most frequently detected immunoreactant in DIF
C3
Agents Used in the Treatment of Cutaneous Necrotizing Venulitis
