Flashcards in Bullous Diseases Deck (27)
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1
Autoimmune, blistering skin disease
IgG mediated
bullous pemphigoid
2
What happens in bullous pemphigoid?
Autoantibodies bind to skin basement membrane
Creates inflammatory reactions that creates blisters
3
What is the epi of bullous pemphigoid?
uncommon, 5th-7th decage = avg age 65
4
What are the triggers of bullous pemphigoid?
UV irradiation
Xray therapy
Drugs- furosemide, ibuprofen, captopril, antibiotics (penicillamine
5
What is the clinical presenation of bullous pemphigoid?
Widespread, tense blisters
Associated with prodromal eruption
6
What are some common sites of bullous pemphigoid?
Axillae, medial thighs, groin, abdomen, forearms- flexor surfaces
Lower legs- often first site
Mucous Membranes- rare
7
What PE do you do for bullous pemphigoid?
Preceding lesion
Weeks to months prior
Various sizes
Serous or hemorrhagic fluid
8
What is the workup for bullous pemphigoid?
biopsy with DIF studies, ELISA
9
What is the treatment for bullous pemphigoid?
Glucocorticoids
Prednisone
Immunosuppressants
Azathioprine (Imuran)
Biologics
Rituximab (Rituxan)
Treat secondary infection
10
What is the prognosis of bullous pemphigoid?
long term remssion after tx
11
What is pemphigus?
Autoimmune
IgG bind to desmogleins (cell to cell adhesions)
Keratinocytes can’t bind leading to split in epidermis/mucosal epithelium
12
What are the two types of pemphigus?
vulgaris
foliaceus
13
What is PV?
serous fliud filled vesicles and bulla affecting oral mucosa
14
How does PV present?
painful
15
What is the sign for PV?
Nikolsky sign
16
What are the sites you get PV?
scalp, face, chest, axillae, groin, umbilicus
17
What is vegetative pemphigus vulgaris?
PV lesions from vegetating granulations
18
What is PF?
Bullae transform to scaly, crusted erosions
No mucosal lesions
Erythematosus base
19
What are common sites for PF?
face, scalp, upper trunk
20
What are teh variations of pemphigus foliaceus?
Pemphigus erythematosus
Pemphigus herpetiformis
Endemic pemphigus foliaceus
IgA pemphigus foliaceus
Drug induced pemphigus foliaceus
21
What is the work up for pemphigus?
Direct immunofluorescence (DIF)- autoantibodies
Indirect immunofluorescence
ELISA
22
What is the treatment for pemphigus?
Need to be aggressive
Glucocorticoids
Prednisone 2-3 mg/kg daily until new blister formation stops
Immunosuppressive therapy
Methotrexate, Rituximab
Antibiotics- if indicated
Wound care
Cleansing baths; dressing changes; Fluid/electrolyte balance
23
What is dermatitis herpetiformis?
Autoimmune- IgA deposits in dermis
Associated with gluten-sensitive enteropathy
24
What do the lesions in dermatitis herpetiformis?
Grouped erythematous papules or wheals
Topped with vesicles
Excoriation and crusts
Extremely pruritic
Burning, stinging, itching before lesions appear
25
What is the epi of dermatitis herpetiformis?
11/100K, men more, 2-4th decade
26
What is the work up for DH?
skin biopsy
27
