Bullae and Desquamation Disorders Flashcards
1
Q
structures that work to adhere cells to one another
A
Desmosomes
2
Q
proteins inside desmosomes to allow for normal desmosome formation
A
Desmogleins
3
Q
what holds the epidermis together?
A
Hemidesmosomes join cells of the epidermal basement membrane to the basilar membrane
4
Q
- Autoimmune disorder
- Loss of cell to cell adhesion in the epidermis
- circulating antibodies of IgG that bind to desmogleins transmembrane glycoproteins in the desmosomes
- Autoantibodies interfere with adhesion = acantholysis
A
pemphigus
5
Q
2 types of pemphigus
A
- Pemphigus Vulgaris: flaccid blisters on skin and erosions on mucous membranes
- Pemphigus Foliaceus: scaly and crusted skin lesions
6
Q
pemphigus MC in who?
A
- Pemphigus Vulgaris - jewish/mediterranean
- Pemphigus Foliaceus - brazil
- 40-60 years old
- M:F = incidence
7
Q
- Vesicles and bullae w/ serous content
- Flaccid/easily ruptured weeping
- Arising on normal skin
- Scattered & discrete - Localized or generalized with random pattern; Extensive erosions = bleed easily
- Nikolsky Sign
A
pemphigus
8
Q
Dislodging or normal appearing epidermis by lateral finger pressure in the vicinity of lesions
Pressure on bulla = lateral extension
what is this sign?
MC areas?
A
Nikolsky Sign
- Scalp
- Face
- Chest
- Axillae
- Groin
- Umbilicus
9
Q
s/s of pemphigus vulgaris
A
- Starts in oral mucosa
- Months may elapse before actual skin lesions
- burning/pain, No pruritus
- Painful mouth lesions = inadequate food intake
- Epistaxis
- Hoarseness
- Dysphagia
- Weakness
- Malaise
- Weight loss
10
Q
s/s of pemphigus foliaceus
A
- No mucosal involvement
- Cutaneous lesions
- initial lesions: flaccid bullae, quickly ruptures, leaving superficial erosion
- MC sites: face, scalp, upper chest, abdomen
11
Q
w/u for pemphigus
A
-
bx of edge of blister: (+) deposits of IgG
- Direct immunofluorescence (IF) staining: IgG and C3 deposited in lesional and paralesional skin; performed on normal-appearing skin adjacent to a lesion - ELISA for IgG ab to desmogelin 1 & 2
- PF +a-Dsg1
- PF + a-Dsg3 and Dsg 1
12
Q
tx for pemphigus
A
- prednisone until no new blisters or nikolsky
- Concomitant Immunosuppressive: azathioprine / mycophenolate mofetil (Cellcept)
- wound care: wet compressions, routine bathing, anticipate infection
13
Q
complications of pemphigus
A
- Fluid and electrolyte imbalances
- Secondary bacterial infections
- Osteoporosis - Calcium/Vit D supplementation
14
Q
- Rare autoimmune disease MC in elderly pts (60-80)
- Interaction of autoantibody with BP antigen
- MC bullous autoimmune disease
A
bullous pemphigoid
15
Q
pathophys of bullous pemphigoid
A
- BPAG1 & BPAG2 In hemidesmosomes of basal keratinocytes
- Followed by complement and mast cell activation, attraction of neutrophils and eosinophils, release multiple bioactive molecules from inflammatory cells