Bullous Pemphigoid

Question 1

What age group does it usually occur?

Answer 1

elderly patients

Question 2

Yearly mortality varies from?

Answer 2

6% to 40%

Question 3

what lesions are usually present?

Answer 3

pruritic urticarial lesions and tense large blisters

Question 4

skin pathology

Answer 4

subepidermal blisters with eosinophils

Question 5

DIF shows?

Answer 5

Immunoglobulin IgG and C3 at epidermal basement membrane of perilesional skin

Question 6

Indirect immunofluorescence

Answer 6

IgG antibasement membrane autoantibodies in the serum

Question 7

what are the proteins of the keratinocyte hemidesmosome?

Answer 7

the autoantigens BPAg1e and the BP180

Question 8

what is basal cell basement membrane adhesion structure?

Answer 8

hemidesmosome

Question 9

What is the therapy of bullous pemphigoid?

Answer 9

topical and systemic corticosteroids and immunosuppresives

Question 10

Bullous pemphigoid typically occurs in patients?

Answer 10

60 years of age, with a peak incidence in the 70s

Question 11

there are several reports of bullous pemphigoid in infants and children, although this is rare

Answer 11

there are several reports of bullous pemphigoid in infants and children, although this is rare

Question 12

what are the hallmarks of bullous pemphigoid?

Answer 12

1.presence of subepidermal blisters
2.lesional and perilesional polymorphonuclear cell infiltrates in the upper dermis
3.IgG autoantibodies and C3 bound to the dermal epidermal junction

Question 13

classic form of bullous pemphigoid

Answer 13

large, tense blisters arising on normal skin or on an erythematous base

Question 14

Lesions are most commonly found on?

Answer 14

1. Flexural surfaces
   2.Lower abdomen
2. Thighs
   although they may occur anywhere

Question 15

Are the Nikolsky and Asboe-Hansen signs

Answer 15

negative

Question 16

Eroded skin from ruptured blisters usually heals spontaneously without scarring, although MILIA can sometimes occur

Answer 16

Eroded skin from ruptured blisters usually heals spontaneously without scarring, although milia can sometimes occur

Question 17

once the lesions heal they may leave hyperpigmented patches that may last for several months

Answer 17

once the lesions heal they may leave hyperpigmented patches that may last for several months

Question 18

Pruritus may be intense in some patients, but minimal in others

Answer 18

Pruritus may be intense in some patients, but minimal in others

Question 19

What other subepidermal blistering diseases also show autoimmune responses to BP180?

Answer 19

1. Pemphigoid gestationis (or herpes gestationis)
   2.cicatricial pemphigoid (or mucous membrane pemphigoid)
   3.linear IgA bullous dermatosis
   4.Lichen planus pemphigoid

Question 20

what are the causes of bullous pemphigoid?

Answer 20

1. most cases occur sporadically without any obvious precipitating factors
   2.trigerred by UV light (either UVB or following PUVA therapy, and radiation therapy)

Question 21

What are the medications associated with the development of bullous pemphigoid?

Answer 21

1. Penicillamine
2. Efalizumab
   3.Etanercept
   4.Furosemide

Question 22

What are the first manifestation of bullous pemphigoid in almost half of patients?

Answer 22

Nonbullous lesions

Question 23

urticarial type lesions precede the more classic tense bullae

Answer 23

urticarial type lesions precede the more classic tense bullae

Question 24

the erythematous component in some bullous pemphigoid patients may appear
1. eczematoid
2. serpiginous
3.targetoid with erythema multiforme-like lesions

Answer 24

the erythematous component in some bullous pemphigoid patients may appear
1. eczematoid
2.serpiginous
3.targetoid with erythema multiforme-like lesions

Question 25

mucous membrane lesions occur in approximately what percentage of patients?

Answer 25

10 percent

Question 26

mucous membrane lesions are almost always limited to what?

Answer 26

oral mucous membranes, particularly the buccal mucosa

Question 27

intact oral mucosa blisters are rare, with erosions more commonly seen

Answer 27

intact oral mucosa blisters are rare, with erosions more commonly seen

Question 28

the lesions in BP heal without scarring and are fairly limited

Answer 28

the lesions in BP heal without scarring and are fairly limited

Question 29

Unlike erythema multiforme, the vermillion border of the lips is rarely involved

Answer 29

Unlike erythema multiforme, the vermillion border of the lips is rarely involved

Question 30

there are rare reports of esophageal involvement in BP

Answer 30

there are rare reports of esophageal involvement in BP

Question 31

the presence of scarring is more suggestive of what?

Answer 31

cicatricial pemphigoid

Question 32

Most cases of bullous pemphigoid occur sporadically?

Answer 32

without any obvious precipitating factors

Question 33

bullous pemphigoid appears to be triggered by?

Answer 33

ultraviolet light, either UVB or following PUVA therapy and radiation therapy

Question 34

What are the medications associated with the development of bullous pemphigoid?

Answer 34

1. penicillamine
   2.efalizumab
   3.etanercept
   4.furosemid

Question 35

The classic form of bullous pemphigoid is characterized by?

Answer 35

large, tense blisters arising on normal skin or on an erythematous base

Question 36

lesions of bullous pemphigoid are most commonly found on what?

Answer 36

flexural surfaces, the lower abdomen, and thighs

Question 37

bullae of bullous pemphigoid are typically filled with?

Answer 37

serous fluid, but may be hemorrhagic

Question 38

The nikolsky and asboe are positive or negative in bullous pemphigoid?

Answer 38

negative

Question 39

eroded skin from ruptured blisters usually heals spontaneously with?

Answer 39

WITHOUT SCARRING, although milia can sometimes occur

Question 40

once the lesions heal for bullous pemphigoid?

Answer 40

they leave hyperpigmented patches that may last for several months

Question 41

pruritus may be intense in some patients, but minimal in others

Answer 41

pruritus may be intense in some patients but may be minimal in others

Question 42

what are the first manifestation of bullous pemphigoid in almost half of patients?

Answer 42

nonbullous lesions; urticarial type lesions precede the more classic tense bullae, and patients may present with these lesions early in the course of disease

Question 43

the erythematous component in some bullous pemphigoid patients may appear?

Answer 43

eczematoid; serpiginous, or targetoid with erythema multiforme-like lesions