Chap 186 Behcet Disease Flashcards
____ is classified as a primary variable vessel vasculitis ,
emphasizing the involvement of any size and type (arterial, venous) of blood
vessel. It is also recognized as an autoinflammatory disease.
Behçet disease (BD)
Behçet disease has a high prevalence in countries along the ___
Silk Road
Onset in children is ___ yr of age
8-12
gene in which BD is markedly associated
HLA-B5101
A number of
infectious agents have been implicated and include ___
streptococci, herpes simplex
virus type 1, and parvovirus B19.
course of BD is characterized by
exacerbations and remissions
mean age of the first symptom is
between 8 and 12 yr
The most frequent
initial symptom is a
painful oral ulcer
description of oral ulcer of BD
oral ulcers are often
recurrent, may be single or multiple, range from 2-10 mm, and may be in anylocation in the oral cavity. They are often very painful. The oral ulcers last 3-10
days and heal without scarring
eye invlovment of BD
anterior uveitis, retinal vascultiis
skin lesions of BD range from
erythema nodosum (seen in approximately
50% of patients) to papulopustular acneiform lesions (85%), folliculitis, purpura,
and ulcers.
___ (seen in 50%) is another skin feature associated with BD
and is a pustular reaction occurring 24-48 hr after a sterile needle puncture or
saline injection; it is not pathognomonic of BD
Pathergy
____is the most common CNS manifestation in children
Dural sinus thrombosis
International Study Group for Behçet Disease (ISG) criteria are most
widely used and require the presence of
oral ulcers (at least 3 times per year) along with 2 other major features, including genital ulcers, a positive pathergy test, uveitis, and the characteristic skin lesions
is highly recommended to treat inflammatory eye disease
Azathioprine