Bullous Diseases

Question 1

Autoimmune, blistering skin disease
IgG mediated

Answer 1

bullous pemphigoid

Question 2

What happens in bullous pemphigoid?

Answer 2

Autoantibodies bind to skin basement membrane
Creates inflammatory reactions that creates blisters

Question 3

What is the epi of bullous pemphigoid?

Answer 3

uncommon, 5th-7th decage = avg age 65

Question 4

What are the triggers of bullous pemphigoid?

Answer 4

UV irradiation
Xray therapy
Drugs- furosemide, ibuprofen, captopril, antibiotics (penicillamine

Question 5

What is the clinical presenation of bullous pemphigoid?

Answer 5

Widespread, tense blisters
Associated with prodromal eruption

Question 6

What are some common sites of bullous pemphigoid?

Answer 6

Axillae, medial thighs, groin, abdomen, forearms- flexor surfaces
Lower legs- often first site
Mucous Membranes- rare

Question 7

What PE do you do for bullous pemphigoid?

Answer 7

Preceding lesion
Weeks to months prior
Various sizes
Serous or hemorrhagic fluid

Question 8

What is the workup for bullous pemphigoid?

Answer 8

biopsy with DIF studies, ELISA

Question 9

What is the treatment for bullous pemphigoid?

Answer 9

Glucocorticoids
Prednisone
Immunosuppressants
Azathioprine (Imuran)
Biologics
Rituximab (Rituxan)
Treat secondary infection

Question 10

What is the prognosis of bullous pemphigoid?

Answer 10

long term remssion after tx

Question 11

What is pemphigus?

Answer 11

Autoimmune
IgG bind to desmogleins (cell to cell adhesions)
Keratinocytes can’t bind leading to split in epidermis/mucosal epithelium

Question 12

What are the two types of pemphigus?

Answer 12

vulgaris
foliaceus

Question 13

What is PV?

Answer 13

serous fliud filled vesicles and bulla affecting oral mucosa

Question 14

How does PV present?

Answer 14

painful

Question 15

What is the sign for PV?

Answer 15

Nikolsky sign

Question 16

What are the sites you get PV?

Answer 16

scalp, face, chest, axillae, groin, umbilicus

Question 17

What is vegetative pemphigus vulgaris?

Answer 17

PV lesions from vegetating granulations

Question 18

What is PF?

Answer 18

Bullae transform to scaly, crusted erosions
No mucosal lesions
Erythematosus base

Question 19

What are common sites for PF?

Answer 19

face, scalp, upper trunk

Question 20

What are teh variations of pemphigus foliaceus?

Answer 20

Pemphigus erythematosus
Pemphigus herpetiformis
Endemic pemphigus foliaceus
IgA pemphigus foliaceus
Drug induced pemphigus foliaceus

Question 21

What is the work up for pemphigus?

Answer 21

Direct immunofluorescence (DIF)- autoantibodies
Indirect immunofluorescence
ELISA

Question 22

What is the treatment for pemphigus?

Answer 22

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

Question 23

What is dermatitis herpetiformis?

Answer 23

Autoimmune- IgA deposits in dermis
Associated with gluten-sensitive enteropathy

Question 24

What do the lesions in dermatitis herpetiformis?

Answer 24

Grouped erythematous papules or wheals
Topped with vesicles
Excoriation and crusts
Extremely pruritic
Burning, stinging, itching before lesions appear

Question 25

What is the epi of dermatitis herpetiformis?

Answer 25

11/100K, men more, 2-4th decade

Question 26

What is the work up for DH?

Answer 26

skin biopsy

Question 27

What is the treatment for DH?

Answer 27

Avoid gluten
Diaminodiphenyl (Dapsone)
sulfapyridine