Bullae and Desquamation Disorders - Exam 2 Flashcards
_____ are structures that work to adhere cells to one another
desmosomes= hold cells together
What hold the epidermis together?
Hemidesmosomes join cells of the epidermal basement membrane to the basilar membrane
_____ are proteins inside desmosomes to allow for normal desmosome formation
desmogleins
_____ 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?
PEMPHIGUS
IgG
Bind to desmogleins transmembrane glycoproteins in the desmosomes and the autoantibodies interfere with adhesion = acantholysis
What are the two types of pemphigus? What is the MC age of onset? What gender?
Pemphigus Vulgaris
Pemphigus Foliaceus
40-60 years old
M:F = incidence
______ pemphigus: are flaccid blisters on skin and erosions on mucous membranes. What ethnicity?
Pemphigus Vulgaris
MC Jewish/Mediterranean descent
____ pemphigus: scaly and crusted skin lesions. What ethnicity?
Pemphigus Foliaceus
brazilian
pemphigus
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?
pemphigus
Scalp
Face
Chest
Axillae
Groin
Umbilicus
What is the Nikolsky sign?
Dislodging or normal appearing epidermis by lateral finger pressure in the vicinity of lesions
Pressure on bulla = lateral extension
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?
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
PEMPHIGUS VULGARIS
No mucosal involvement
Cutaneous lesions
flaccid bullae, quickly ruptures, leaving superficial erosion
What am I?
Where are the MC sites?
PEMPHIGUS FOLIACEUS
face, scalp, upper chest, abdomen
What is the best test for pemphigus? Where do you do it? What will it show?
bx: best performed at edge of a blister
results: (+) deposits of IgG
What does a Direct immunofluorescence (DIF) staining reveal in pemphigus? Where should it be performed?
IgG and often C3 deposited in lesional and paralesional skin
DIF should be performed on normal-appearing skin adjacent to a lesion
What will ELISA show for a pt with PEMPHIGUS FOLIACEUS? PEMPHIGUS VULGARIS?
PF: +a-Dsg1
PV: +a-Dsg3 and +a-Dsg 1
What is the tx for pemphigus? What should you do next?
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
What are the complications of pemphigus? Need to combat with ____
Fluid and electrolyte imbalances
Secondary bacterial infections
Osteoporosis
combat osteoporosis with calcium and vit D supplementation
______ is a rare autoimmune disease of the skin usually seen in elderly patients due to the interaction of autoantibody with BP antigen
BULLOUS PEMPHIGOID
BPAG1 & BPAG2 In hemidesmosomes of basal keratinocytes are associated with _____. What happens next?
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
What is the MC age of onset? What sex is MC?
60-80 years old
male and female equal rates of occurence
_____ is the MC bullous autoimmune disease
BULLOUS PEMPHIGOID
What am I?
What will the pt complain of?
positive or negative Nikolsky sign?
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
What are the MC sites for bullous pemphigoid? Can they involve the mouth?
axilla, medial thigh, groin, abdomen, ventral forearm, lower legs
YES: Oral lesions will be less severe and less painful than pemphigus
**What is the gold standard test for bullous pemphigoid? What will you see?
bx perilesional skin
Linear IgG deposits along the basement membrane
C3 may occur in the absence of IgG
What will ELISA show in bullous pemphigoid? What is important to note?
BPAG1 and BPAG2
few labs will perform this test, so need to confirm with the lab before you order it
What is the tx for bullous pemphigoid?
REFER TO DERM!
prednisone +/-Azathioprine
topical steroids may be helpful
_____ is an acute hypersensitivity reaction affecting the skin and mucous membranes. What is the MC cause? What additional organ system can be involved?
ERYTHEMA MULTIFORME
HSV
eye involvement
ERYTHEMA MULTIFORME
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?
ERYTHEMA MULTIFORME
Usually bilateral and symmetrical
What are the criteria for ERYTHEMA MULTIFORME minor?
Little - no mucosal involvement
(+) vesicle, (-) bullae
no systemic symptoms
confined to extremities & face
What are the criteria for ERYTHEMA MULTIFORME major? What is the MC etiology?
mucosal involvement
confluence of lesions
(+) Nikolsky sign
(+) constitutional symptoms
cheilitis/stomatitis
vulvitis/balanitis
eye involvement
drug reaction
How do you dx ERYTHEMA MULTIFORME?
clinical dx off hx and PE
What is the tx for ERYTHEMA MULTIFORME minor?
What is the tx for erythema multiforme major?
What is the tx for erythema multiforme that is recurrent? What are the 2 complications?
Daily prophylactic antiviral therapy
Secondary bacterial infection
Fluid/electrolyte imbalances
ERYTHEMA MULTIFORME
_____ and ____ are cytotoxic event caused by an immune response results in destruction of keratinocyte. What are the causes? What is the MC?
SJS and TEN
drugs-MC
Chemicals
Mycoplasma
Viral infections
Immunization
What am I?
What other s/s will the pt complain of?
Fever
Chills
Malaise
Headache
Sore throat
Nausea
Vomiting
Diarrhea
Skin tenderness
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?
SJS/TEN
2 zones of color
Face and extremities
Will SJS/TEN affect the mucosa? Will it affect the hair and nail?
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
What is considered an emergency in SJS/TEN?
Systemic symptoms
Fever
HR >120 BPM
Sloughing of epidermis
How is the dx of SJS/TEN made? **What is the classification system used?
clinical dx but a bx is helpful
**
SJS = <10% body surface
SJS/TEN = 10-30% body surface
TEN = > 30% body surface
**
What is the tx for SJS/TEN?
can be treated like burn pts
