Chapter 16-Derm Dz 4 Flashcards
What is the most common autoimmune blistering condition?
Bullous pemphigoid 10:1,000,000 per year
How does bullous pemphigoid clincally differ from MMP?
BP = remission & relapse VS MMP = protracted & progressive
Bullous pemphigoid is really a condition for which age population? Gender?
older..75-80 years. No gender predilection, but argument that male predominance if taking age into consideration
What % range of bullous pemphigoid patients have oral lesions?
10-20%
Bullous pemphigoid histology: the presence of what cell type within the bulla itself is characteristic?
eosinophils
Bullous pemphigoid DIF shows a continuous
linear band of immunoreactants, usually WHAT 2?, localized to the basement membrane zone in 90% to 100% of affected patients
IgG and C3
Bullous pemphigoid: Which 2 hemidesmosome proteins do IgG and C3 attack? Which layer?
antigens BP180 and BP230…the localization of BP180 to the upper portion of the lamina lucida of the basement membrane
What is the age and sex matched mortality rate for 1 year after dx of bullous pemphigoid?
20%
While EM is generally an uncertain etiology, about 50% of the cases have a precipitating cause like what 3 reasons?
- HSV 2.Mycoplasma pneumoniae 3.drugs (abx, analgesics)
What are the DIF and IIF findings for EM?
nonspecific, but r/o MMP, PV, etc
It was INITIALLY THOUGHT that EM minor = ______ and EM major = ______, but now are distinctly different entities
EM minor = SJS EM major = TEN
Age range and gender for EM
20-30’s, slight female predilection
What is the buzzword for the clinical appearance of EM skin lesions?
targetoid / bulls eye
What % of patients with EM have oral involvement? (wide range)
25-70%
What are the requirements to dx EM major? (2)
1) two or more mucosal sites are affected in conjunction with
2) widespread skin lesions
Aside from the oral cavity, what are the other two commonly involved mucosal sites in EM major?
ocular (can get symblepharon like MMP) or genital
What are 4 features of EM histology?
- Subepithelial or intraepithelial vesiculation
- necrotic basal keratinocytes 3.mixed inflammatory infiltrate is present, consisting of lymphocytes, neutrophils, and often eosinophils 4. Perivascular
EM is considered to be self-limiting, lasting __ - __ weeks, what % of patients get recurrence? What times of year do recurrences typically occur? What is the prognosis?
2-6 weeks, 20% - in the SPRING AND FALL…typically NOT life threatning, unless in most severe form
What is the primary difference between etiology of EM vs SJS / TEN?
EM = HSV most commonly….SJS/TEN ALMOST ALWAYS drug exposure (over 200 meds ID’d)
What differentiates SJS from TEN?
degree of skin involvment: SJS = <10% and TEN = >30%
What are the prevalences for SJS and TEN?
SJS = 1-7:1,000,000…..TEN = 1:1,000,000
Age for SJS? TEN? Gender?
SJS = younger patients TEN = over 60 years…female predilection
What are the prodromal symptoms for SJS/TEN like?
Flu like
What differs in location of the initial lesions in SJS/TEN from EM?
SJS/TEN = flat lesions on trunk