Bullous Diseases Flashcards
Autoimmune, blistering skin disease
IgG mediated
bullous pemphigoid
What happens in bullous pemphigoid?
Autoantibodies bind to skin basement membrane
Creates inflammatory reactions that creates blisters
What is the epi of bullous pemphigoid?
uncommon, 5th-7th decage = avg age 65
What are the triggers of bullous pemphigoid?
UV irradiation
Xray therapy
Drugs- furosemide, ibuprofen, captopril, antibiotics (penicillamine
What is the clinical presenation of bullous pemphigoid?
Widespread, tense blisters
Associated with prodromal eruption
What are some common sites of bullous pemphigoid?
Axillae, medial thighs, groin, abdomen, forearms- flexor surfaces
Lower legs- often first site
Mucous Membranes- rare
What PE do you do for bullous pemphigoid?
Preceding lesion
Weeks to months prior
Various sizes
Serous or hemorrhagic fluid
What is the workup for bullous pemphigoid?
biopsy with DIF studies, ELISA
What is the treatment for bullous pemphigoid?
Glucocorticoids Prednisone Immunosuppressants Azathioprine (Imuran) Biologics Rituximab (Rituxan) Treat secondary infection
What is the prognosis of bullous pemphigoid?
long term remssion after tx
What is pemphigus?
Autoimmune
IgG bind to desmogleins (cell to cell adhesions)
Keratinocytes can’t bind leading to split in epidermis/mucosal epithelium
What are the two types of pemphigus?
vulgaris
foliaceus
What is PV?
serous fliud filled vesicles and bulla affecting oral mucosa
How does PV present?
painful
What is the sign for PV?
Nikolsky sign
What are the sites you get PV?
scalp, face, chest, axillae, groin, umbilicus
What is vegetative pemphigus vulgaris?
PV lesions from vegetating granulations
What is PF?
Bullae transform to scaly, crusted erosions
No mucosal lesions
Erythematosus base
What are common sites for PF?
face, scalp, upper trunk
What are teh variations of pemphigus foliaceus?
Pemphigus erythematosus Pemphigus herpetiformis Endemic pemphigus foliaceus IgA pemphigus foliaceus Drug induced pemphigus foliaceus
What is the work up for pemphigus?
Direct immunofluorescence (DIF)- autoantibodies
Indirect immunofluorescence
ELISA
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
What is dermatitis herpetiformis?
Autoimmune- IgA deposits in dermis
Associated with gluten-sensitive enteropathy
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
What is the epi of dermatitis herpetiformis?
11/100K, men more, 2-4th decade
What is the work up for DH?
skin biopsy
What is the treatment for DH?
Avoid gluten
Diaminodiphenyl (Dapsone)
sulfapyridine
