Blistering Diseases

Question 1

Pemphigus Vulgaris

most common in who?

Answer 1

pts in their 30s and 40s

Question 2

Pemphigus Vulgaris

associated w/ what meds?

4

Answer 2

1. penicillamine
2. captopril
3. cephalosporins
4. phenobarbituals

Question 3

Pemphigus Vulgaris

generally describe

type of disorder, severity, of what?

Answer 3

life-threatening, chronic autoimmune blistering disorder of the mucus membranes & skin

Question 4

Pemphigus Vulgaris

pathophys

Answer 4

1. type 2 hypersensitivity rxn where autoantibodies against desmoglein lead to acantholysis
2. desmoglein connects keratinocytes in the skin

Question 5

Pemphigus Vulgaris

clinical presentation

Answer 5

• initial: painful erosion/ulceration
• develops: painful, flaccid skin bola that ruptures easily
• leaves painful denuded skin erosions that bleed easily

Question 6

Pemphigus Vulgaris

where is the initial lesion most commonly located?

Answer 6

intra orally

Question 7

Pemphigus Vulgaris

what does pos Nikolsky sign mean?

Answer 7

detachment of skin under pressure or trauma

Question 8

Pemphigus Vulgaris

dx

Answer 8

1. punch biopsy
2. ELISA- anti-desmoglein or anti-epithelial autoantibodies

Question 9

Pemphigus Vulgaris

what will you see on punch biopsy?

Answer 9

IgG throughout epidermis
basal keritanocytes in a pattern that resembles a “row of tombstones”

Question 10

Pemphigus Vulgaris

tx

3 components

Answer 10

1. systemic glucocorticoids
2. would care
3. (+/-) rituximab, mycophenolate, or azathioprine

Question 11

Bullous Pemphigoid

primariy seen in?

Answer 11

elderly

Question 12

Bullous Pemphigoid

can be induced by what?

Answer 12

drugs (loop diuretics, metformin)

Question 13

Bullous Pemphigoid

autoimmune disorder that leads to?

Answer 13

• blister formation
• severe itching

Question 14

Bullous Pemphigoid

pathophys

Answer 14

• type 2 hypersensitivity rxn
• IgG autoantibodies against hemidesmosomes & basement membrane zone causing subepidermal blistering

Question 15

Bullous Pemphigoid

clinical presentation

5 components

Answer 15

• Initial: pruritis, eczematous or urticarialerythematous plaques
• progress to: multiple tense large bullae that don’t easily rupture
• mucosal disease (10-30%)
• negative Nikolsky sign
• most commonly found in groin, axilla, trunk, flexural areas of extremities

Question 16

Bullous Pemphigoid

Dx

Answer 16

• punch biopsy w/ direct immunofluorescence
• ELISA

Question 17

Bullous Pemphigoid

what do you see on punch biopsy?

Answer 17

• linear C3, IgG along the dermal-epidermal junction, subepidermal blistering, eosinophilia

Question 18

Bullous Pemphigoid

what do you see on ELISA?

Answer 18

• autoantibodies against BP antigen 230 & 180

Question 19

Bullous Pemphigoid

tx

localized/mild disease vs extensive/severe

Answer 19

• mild: high potency topical corticosteroids + tetracyclines
• severe: systemic corticosteroids

Question 20

Porphyria Cutanea Tarda

general overview

Answer 20

hypersensitivity of the skin to abnormal porphyrins when exposed to light, leading to blistering disease of sun exposed areas

Question 21

Porphyria Cutanea Tarda

caused by which enzyme?

Answer 21

uroporphyrinogen decarboxylase

Question 22

Porphyria Cutanea Tarda

risk factors

5

Answer 22

1. Liver Disease: hepatitis C, alcoholism, hemachromatosis
2. Estrogen use
3. tobacco use

Question 23

Porphyria Cutanea Tarda

clinical presentation

Answer 23

• chronic blistering photosensitivity of sun exposed areas
• can lead to hyper or hypo pigmentation or scarring

Question 24

Porphyria Cutanea Tarda

Dx

Answer 24

• 24 hr collection of urine
• plasma porphyrin profile

Question 25

# Porphyria Cutanea Tarda tx

Answer 25

treat the underlying cause