BULLOUS DISEASES II

Question 1

what are the bulls diseases?

Answer 1

• Pemphigis vulgaris
• Bullous pemphigoid
• Erythema multiforme/SJS
• Dermatitis herpetiformis

Question 2

circumscribed, fluid-containing elevations up to 1 cm in size

Answer 2

Vesicle

Question 3

rounded or irregular shaped blisters

larger than 1 cm

Answer 3

Bullae

Question 4

diagnostic maneuver- put lateral pressure on unblistered skin and
epithelium shears off

Answer 4

Nikolsky’s sign

Question 5

extension of blister to
adjacent normal skin when putting pressure
on top of blister

Answer 5

Absoe-Hansen’s sign

Question 6

what is this?

• Abs – desmoglein 1 or desmoglein 3

• Frequently starts as painful oral bullae and
erosions

• Flaccid, thin-walled bullae rupture, leaving
denuded areas of skin

• High mortality rate prior to corticosteroids
• • Nikolsky and Absoe-Hansen signs

Answer 6

Pemphigus Vulgaris

Question 7

what is this?

• Abs – BPAG1, BPAG2 (type XVII collagen)
• Chronic disease mainly of elderly individuals

• Large, tense bullae (in contrast with flaccid
bullae of pemphigus)

• Oral lesions in 1/3 of cases

Answer 7

Bullous Pemphigoid

non-bulbous phase
bullous phase

Question 8

what is this?

• Most patients are 10-30 year olds
• Highest incidence in winter
• Prodromal symptoms precedes the polymorphous cutaneous eruption

• Multiple triggers: HSV, mycoplasma
pneumoniae, drugs

Answer 8

Erythema Multiforme

Question 9

what is this?

• Elderly more frequently affected
• Rare – 5 cases/million yearly
• Usually caused by medication – exact mechanism unclear

• Most common culprits – ALLOPURINOL, SULF ABX
(BACTRIM), ANTICONVULSANTS, HAART

• SJS - <10% BSA, SJS-TEN – 10-30% BSA, TEN->30% BSA

• Can be targetoid, but lack raised target like EM, involves
mucosa – mouth, eyes, genitals

• Nikoslky & Asboe-Hansen signs +
• Treatment – largely supportive

Answer 9

Stevens-Johnson Syndrome (SJS)

Question 10

what is this?

• Cutaneous manifestation of celliac/gluten sensitivity

• Pruritic grouped vesicles of elbows, interscapular
area and sacral area

• Often no vesicles identified on exam (so pruritic patient has scratched them all)
• Mucous membranes not affected
• Abs – IgA against transglutaminase 3
• Chronic course with relapses and remissions
• Treat with dapsone and gluten free diet

Answer 10

Dermatitis Herpetiformis