Bullae and Desquamation Disorders - Exam 2

Question 1

_____ are structures that work to adhere cells to one another

Answer 1

desmosomes= hold cells together

Question 2

What hold the epidermis together?

Answer 2

Hemidesmosomes join cells of the epidermal basement membrane to the basilar membrane

Question 3

_____ are proteins inside desmosomes to allow for normal desmosome formation

Answer 3

desmogleins

Question 4

_____ is an autoimmune disorder that causes a loss of cell to cell adhesion in the epidermis due to circulating antibodies of the ____ class. What happens next?

Answer 4

PEMPHIGUS

IgG

Bind to desmogleins transmembrane glycoproteins in the desmosomes and the autoantibodies interfere with adhesion = acantholysis

Question 5

What are the two types of pemphigus? What is the MC age of onset? What gender?

Answer 5

Pemphigus Vulgaris

Pemphigus Foliaceus

40-60 years old

M:F = incidence

Question 6

______ pemphigus: are flaccid blisters on skin and erosions on mucous membranes. What ethnicity?

Answer 6

Pemphigus Vulgaris

MC Jewish/Mediterranean descent

Question 7

____ pemphigus: scaly and crusted skin lesions. What ethnicity?

Answer 7

Pemphigus Foliaceus

brazilian

Question 8

Answer 8

pemphigus

Question 9

Vesicles and bullae w/ serous content
Flaccid/easily ruptured weeping
Arising on normal skin
Scattered & discrete: Localized or generalized with random pattern
Extensive erosions will bleed easily

What am I?
Where are the MC areas?

Answer 9

pemphigus

Scalp
Face
Chest
Axillae
Groin
Umbilicus

Question 10

What is the Nikolsky sign?

Answer 10

Dislodging or normal appearing epidermis by lateral finger pressure in the vicinity of lesions

Pressure on bulla = lateral extension

Question 11

Where does pemphigus vulgaris start? How long does it take to see a skin lesion? What will the pt complain of? Will it have a positive or negative Nikolsky sign?

Answer 11

Starts in the oral mucosa

Months may elapse before actual skin lesions

+ burning/pain, No pruritus, but s/s of the pt NOT wanting to eat because it is painful

+ Nikolsky sign

Question 12

Answer 12

PEMPHIGUS VULGARIS

Question 13

No mucosal involvement
Cutaneous lesions
flaccid bullae, quickly ruptures, leaving superficial erosion

What am I?
Where are the MC sites?

Answer 13

PEMPHIGUS FOLIACEUS

face, scalp, upper chest, abdomen

Question 14

What is the best test for pemphigus? Where do you do it? What will it show?

Answer 14

bx: best performed at edge of a blister

results: (+) deposits of IgG

Question 15

What does a Direct immunofluorescence (DIF) staining reveal in pemphigus? Where should it be performed?

Answer 15

IgG and often C3 deposited in lesional and paralesional skin

DIF should be performed on normal-appearing skin adjacent to a lesion

Question 16

What will ELISA show for a pt with PEMPHIGUS FOLIACEUS? PEMPHIGUS VULGARIS?

Answer 16

PF: +a-Dsg1

PV: +a-Dsg3 and +a-Dsg 1

Question 17

What is the tx for pemphigus? What should you do next?

Answer 17

prednisone and azathioprine OR mycophenolate mofetil

prednisone 2-3mg/kg until the pt stops getting new blisters and existing blisters start having a -Nikolsky sign

taper quickly to half dose, then taper to min effective dose

azathioprine or mycophenolate mofetil

wound care: wet compression, routine bathing, anticipate infection

Question 18

What are the complications of pemphigus? Need to combat with ____

Answer 18

Fluid and electrolyte imbalances

Secondary bacterial infections

Osteoporosis

combat osteoporosis with calcium and vit D supplementation

Question 19

______ is a rare autoimmune disease of the skin usually seen in elderly patients due to the interaction of autoantibody with BP antigen

Answer 19

BULLOUS PEMPHIGOID

Question 20

BPAG1 & BPAG2 In hemidesmosomes of basal keratinocytes are associated with _____. What happens next?

Answer 20

BULLOUS PEMPHIGOID

antibodies activate the complement system and recruit inflammatory cells to lead an autoimmune destruction of the basement membrane, weakens adhesion between dermis and epidermis lead to accumulation of serous fluids in the pseudopocket between the epidermis and dermis

Question 21

What is the MC age of onset? What sex is MC?

Answer 21

60-80 years old

male and female equal rates of occurence

Question 22

_____ is the MC bullous autoimmune disease

Answer 22

BULLOUS PEMPHIGOID

Question 23

What am I?
What will the pt complain of?
positive or negative Nikolsky sign?

Answer 23

BULLOUS PEMPHIGOID

will have prodrome of pruritus/urticaria and papular lesions

then large, tense, firm bullae that are painful erosions after rupture

(-) Nikolsky-> they will pop instead

Question 24

What are the MC sites for bullous pemphigoid? Can they involve the mouth?

Answer 24

axilla, medial thigh, groin, abdomen, ventral forearm, lower legs

YES: Oral lesions will be less severe and less painful than pemphigus

Question 25

**What is the gold standard test for bullous pemphigoid? What will you see?

Answer 25

bx perilesional skin Linear IgG deposits along the basement membrane C3 may occur in the absence of IgG

Question 26

What will ELISA show in bullous pemphigoid? What is important to note?

Answer 26

BPAG1 and BPAG2 few labs will perform this test, so need to confirm with the lab before you order it

Question 27

What is the tx for bullous pemphigoid?

Answer 27

REFER TO DERM! prednisone +/-Azathioprine topical steroids may be helpful

Question 28

_____ is an acute hypersensitivity reaction affecting the skin and mucous membranes. What is the MC cause? What additional organ system can be involved?

Answer 28

ERYTHEMA MULTIFORME HSV eye involvement

Question 29

Answer 29

ERYTHEMA MULTIFORME

Question 30

Erythematous, papular, or urticarial type lesion May precede bullae formation by months Contain serous or hemorrhagic fluid Localized or generalized Pruritic and painful Bullae: can be small or large Tense, firm topped oval or round Fever, weakness, malaise, and fatigue What am I? What is the distribution?

Answer 30

ERYTHEMA MULTIFORME Usually bilateral and symmetrical

Question 31

What are the criteria for ERYTHEMA MULTIFORME minor?

Answer 31

Little - no mucosal involvement (+) vesicle, (-) bullae no systemic symptoms confined to extremities & face

Question 32

What are the criteria for ERYTHEMA MULTIFORME major? What is the MC etiology?

Answer 32

mucosal involvement confluence of lesions (+) Nikolsky sign (+) constitutional symptoms cheilitis/stomatitis vulvitis/balanitis eye involvement drug reaction

Question 33

How do you dx ERYTHEMA MULTIFORME?

Answer 33

clinical dx off hx and PE

Question 34

What is the tx for ERYTHEMA MULTIFORME minor?

Answer 34

Question 35

What is the tx for erythema multiforme major?

Answer 35

Question 36

What is the tx for erythema multiforme that is recurrent? What are the 2 complications?

Answer 36

Daily prophylactic antiviral therapy Secondary bacterial infection Fluid/electrolyte imbalances

Question 37

Answer 37

ERYTHEMA MULTIFORME

Question 38

_____ and ____ are cytotoxic event caused by an immune response results in destruction of keratinocyte. What are the causes? What is the MC?

Answer 38

SJS and TEN drugs-MC Chemicals Mycoplasma Viral infections Immunization

Question 39

What am I? What other s/s will the pt complain of?

Answer 39

Fever Chills Malaise Headache Sore throat Nausea Vomiting Diarrhea Skin tenderness

Question 40

Abrupt onset of mucocutaneous lesions Macule, papule, central vesicle/bullae, erosions Rapid confluence +Nikolsky sign full thickness epidermal detachment What am I? the target lesion will have ____ of color What is the distribution?

Answer 40

SJS/TEN 2 zones of color Face and extremities

Question 41

Will SJS/TEN affect the mucosa? Will it affect the hair and nail?

Answer 41

YES!! very painful, erythematous erosions on lips, buccal mucosa, conjunctiva, genitalia, anal region YES! can also result in loss of lashes and nails in TEN

Question 42

What is considered an emergency in SJS/TEN?

Answer 42

Systemic symptoms Fever HR >120 BPM Sloughing of epidermis

Question 43

How is the dx of SJS/TEN made? **What is the classification system used?

Answer 43

clinical dx but a bx is helpful ** SJS = <10% body surface SJS/TEN = 10-30% body surface TEN = > 30% body surface **

Question 44

What is the tx for SJS/TEN?

Answer 44

can be treated like burn pts