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
What percent of patients with SJS/TEN have mucosal involvment?
nearly all (99%? lol)…particularly the oral mucosa
What is a promising new treatment for SJS/TEN? What are their mortality rates?
First stop the drug, dont tx with corticosteriods, and then IV pooled immunoglobulins block the Fas ligand….TEN = 25-30% and SJS = 1-5%.
Get it. What are the 5 alternate names for God Damn Erythema Migrans?
- Geographic Tongue 2.Benign Migratory Glossitis 3.Wandering rash of the tonuge 4. Erythema Areata Migrans 5. Stomatitis Areata Migrans
What is the prevelence for geographic tongue? Gender?
1-3% of the population…2:1 female (controversial, some studies show no predilection, but really, who cares?)
Which papillae atrophy in geo tongue to cause the erythema?
Filliform papillae
What other condition of the tongue is associated with erythema migrans in 1/3 of cases?
fissured tongue
Geo tongue histo: term for pattern seen? Also, Buzzword for the abscesses?
Psoriasiform mucositis….MUNRO abscesses
What is the HLA group associated with erythema migrans?
HLA-Cw6
What is the alternate name for Reactive Arthritis?
Reiter syndrome
What is the triad for reactive arthritis?
- Nongonococcal urethritis 2.Arthritis 3.Conjunctivis
Age and gender for reactive arthritis?
Young adult men (9:1 ratio)
What is the HLA associated with Reactive arthritis? What does RA usually develop after?
HLA-B27…1-4 weeks following dysentery or venereal disease
What fraction of patients with Reactive arthritis have TMJ involvment?
1/3
What is the term for the Reactive arthritis lesion found on the glans penis? What oral lesion does this resemble clinically?
Balanitis Circinata….geographic tongue
What skin condition does Reactive arthritis resemble on histology?
psoriasis
