Block 1- Bullous disorders Flashcards
Vesicles are ___ blister
small
bull are ___ blister
LARGE
name disease:
A potentially life-threatening autoimmune mucocutaneous disease - mortality rate of approximately 5-15%
Pemphigus vulgaris
before ______, Pemphigus vulgaris
was fatal
glucocorticoids
name disease:
- intraepidermal, blistering disease
- affects skin and mucous membranes
- mediated by circulating autoantibodies directed autoantibodies against the connections between epidermal cells
Pemphigus vulgaris
intrapidermal blister is associated with PV or BP?
Pemphigus vulgaris
Pemphigus vulgaris is mediated by circulating autoantibodies directed autoantibodies against the connections between ____ cells
epidermal
Epidemiology of Pemphigus vulgaris
Age: Younger patient- <60 yrs (vs. Bullous pemphigoid >60 yrs)
Sex: Male=Female
Pemphigus vulgaris CLINICAL COURSE
First: Painful oral erosions occur 1st (NOT Pruritic)
Next: skin blisters – may be delayed weeks or months
do they bullae of Pemphigus vulgaris easily rupture?
yes
does Pemphigus vulgaris have a positive or negative Nikolsy’s sign?
positive
what does it mean when there is a positive Nikolsy’s sign in Pemphigus vulgaris?
Application of pressure to small intact bullae causes the fluid to dissect laterally into the midepidermal areas
most common sites where your find Pemphigus vulgaris
o Oral cavity (other mucosal sites later in course)
o scalp
o face
o axillae
how to diagnose Pemphigus vulgaris?
- Skin Biopsy [Bx]
- Immunofluorescence of PERILESIONAL skin [in Michel’s medium]
treatment for Pemphigus vulgaris?
CORTICOSTEROIDS AND IMMUNOSUPPRESSIVE AGENTS
is remission of Pemphigus vulgaris possible?
yes
in about 75% of pts, you can discontinue treatment of Pemphigus vulgaris in
__ years.
10
Differential Diagnosis for pemphigus vulgaris
- bullous pemphigoid (think elderly)
- epidermolysis bullosa
- dermatitis herpetiformis [gluten]
- bullous systemic lupus erythematosus
- bullous drug eruptions
prognosis for pemphigus vulgaris
- Tripled mortality rate vs unaffected population
- Complications secondary to the use of high-dose corticosteroids contribute to the mortality rate
- Morbidity and mortality are related to the extent of disease, the maximum dose of systemic steroids required to induce remission, and the presence of other diseases
- Prognosis worse with extensive pemphigus vulgaris and in older aged patients