Chapter 16-Derm Dz 4

Question 1

What is the most common autoimmune blistering condition?

Answer 1

Bullous pemphigoid 10:1,000,000 per year

Question 2

How does bullous pemphigoid clincally differ from MMP?

Answer 2

BP = remission & relapse VS MMP = protracted & progressive

Question 3

Bullous pemphigoid is really a condition for which age population? Gender?

Answer 3

older..75-80 years. No gender predilection, but argument that male predominance if taking age into consideration

Question 4

What % range of bullous pemphigoid patients have oral lesions?

Answer 4

10-20%

Question 5

Bullous pemphigoid histology: the presence of what cell type within the bulla itself is characteristic?

Answer 5

eosinophils

Question 6

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

Answer 6

IgG and C3

Question 7

Bullous pemphigoid: Which 2 hemidesmosome proteins do IgG and C3 attack? Which layer?

Answer 7

antigens BP180 and BP230…the localization of BP180 to the upper portion of the lamina lucida of the basement membrane

Question 8

What is the age and sex matched mortality rate for 1 year after dx of bullous pemphigoid?

Answer 8

20%

Question 9

While EM is generally an uncertain etiology, about 50% of the cases have a precipitating cause like what 3 reasons?

Answer 9

1. HSV 2.Mycoplasma pneumoniae 3.drugs (abx, analgesics)

Question 10

What are the DIF and IIF findings for EM?

Answer 10

nonspecific, but r/o MMP, PV, etc

Question 11

It was INITIALLY THOUGHT that EM minor = ______ and EM major = ______, but now are distinctly different entities

Answer 11

EM minor = SJS EM major = TEN

Question 12

Age range and gender for EM

Answer 12

20-30’s, slight female predilection

Question 13

What is the buzzword for the clinical appearance of EM skin lesions?

Answer 13

targetoid / bulls eye

Question 14

What % of patients with EM have oral involvement? (wide range)

Answer 14

25-70%

Question 15

What are the requirements to dx EM major? (2)

Answer 15

1) two or more mucosal sites are affected in conjunction with
2) widespread skin lesions

Question 16

Aside from the oral cavity, what are the other two commonly involved mucosal sites in EM major?

Answer 16

ocular (can get symblepharon like MMP) or genital

Question 17

What are 4 features of EM histology?

Answer 17

1. Subepithelial or intraepithelial vesiculation
2. necrotic basal keratinocytes 3.mixed inflammatory infiltrate is present, consisting of lymphocytes, neutrophils, and often eosinophils 4. Perivascular

Question 18

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?

Answer 18

2-6 weeks, 20% - in the SPRING AND FALL…typically NOT life threatning, unless in most severe form

Question 19

What is the primary difference between etiology of EM vs SJS / TEN?

Answer 19

EM = HSV most commonly….SJS/TEN ALMOST ALWAYS drug exposure (over 200 meds ID’d)

Question 20

What differentiates SJS from TEN?

Answer 20

degree of skin involvment: SJS = <10% and TEN = >30%

Question 21

What are the prevalences for SJS and TEN?

Answer 21

SJS = 1-7:1,000,000…..TEN = 1:1,000,000

Question 22

Age for SJS? TEN? Gender?

Answer 22

SJS = younger patients TEN = over 60 years…female predilection

Question 23

What are the prodromal symptoms for SJS/TEN like?

Answer 23

Flu like

Question 24

What differs in location of the initial lesions in SJS/TEN from EM?

Answer 24

SJS/TEN = flat lesions on trunk

Question 25

What percent of patients with SJS/TEN have mucosal involvment?

Answer 25

nearly all (99%? lol)…particularly the oral mucosa

Question 26

What is a promising new treatment for SJS/TEN? What are their mortality rates?

Answer 26

First stop the drug, dont tx with corticosteriods, and then IV pooled immunoglobulins block the Fas ligand….TEN = 25-30% and SJS = 1-5%.

Question 27

Get it. What are the 5 alternate names for God Damn Erythema Migrans?

Answer 27

1. Geographic Tongue 2.Benign Migratory Glossitis 3.Wandering rash of the tonuge 4. Erythema Areata Migrans 5. Stomatitis Areata Migrans

Question 28

What is the prevelence for geographic tongue? Gender?

Answer 28

1-3% of the population…2:1 female (controversial, some studies show no predilection, but really, who cares?)

Question 29

Which papillae atrophy in geo tongue to cause the erythema?

Answer 29

Filliform papillae

Question 30

What other condition of the tongue is associated with erythema migrans in 1/3 of cases?

Answer 30

fissured tongue

Question 31

Geo tongue histo: term for pattern seen? Also, Buzzword for the abscesses?

Answer 31

Psoriasiform mucositis….MUNRO abscesses

Question 32

What is the HLA group associated with erythema migrans?

Answer 32

HLA-Cw6

Question 33

What is the alternate name for Reactive Arthritis?

Answer 33

Reiter syndrome

Question 34

What is the triad for reactive arthritis?

Answer 34

1. Nongonococcal urethritis 2.Arthritis 3.Conjunctivis

Question 35

Age and gender for reactive arthritis?

Answer 35

Young adult men (9:1 ratio)

Question 36

What is the HLA associated with Reactive arthritis? What does RA usually develop after?

Answer 36

HLA-B27…1-4 weeks following dysentery or venereal disease

Question 37

What fraction of patients with Reactive arthritis have TMJ involvment?

Answer 37

1/3

Question 38

What is the term for the Reactive arthritis lesion found on the glans penis? What oral lesion does this resemble clinically?

Answer 38

Balanitis Circinata….geographic tongue

Question 39

What skin condition does Reactive arthritis resemble on histology?

Answer 39

psoriasis