Immunbullous Disease - Schwarzenberger

Question 1

Epidemiology of Bullous pemphigoid?

Answer 1

Affects older people, 68-82; Women predominant

Question 2

What is the pathophysiology of bullous pemphigoid?

Answer 2

Autoantibodies against the hemidesmosomes in the basal keratinocytes –> BP 180 and BP 230 proteins

Question 3

Describe the lesions seen EARLY in bullous pemphigoid

Answer 3

very itchy, urticarial rash

Question 4

Describe the blisters of bullous pemphigoid. Where are they located?

Answer 4

Tense (stay taut with pressure), normally symmetric in distribution. Might have erythema

Question 5

Picture of bullous pemphigoid

Answer 5

Question 6

Mucosal involvement in bullous pemphigoid?

Answer 6

20% of cases; eye involvement can cause blindness, oral lesions common

Question 7

What is gestational pemphigoid? What risks are associatesd with it?

Answer 7

Bullous pemphigoid arising in a pregnant woman; can cause preterm labor or low weight babies

Question 8

What will skin biopsy of bullous pemphigoid show when immunofluoresced

Answer 8

IgG and C3 deposition at the dermal-epidermal junction (LINEAR PATTERN)

Question 9

Bullous Pemphigoid and antibodies in blood?

Answer 9

May or may not find antibodies in the blood

Question 10

What is the treatment for bullous pemphigoid?

Answer 10

Long term systemic steroids and/or immunosuppresive Rx; requires aggressive tx

Question 11

Epidemiology of Pemphigus vulgaris?

Answer 11

All ages (mean onset 50-60); male=female; incdience higher in Ashkenazi Jews and those of Mediterranean descent

Question 12

Pathophysiology of Pemphigus Vulgaris

Answer 12

Autoantibodies against the desmoglein 1 and 3

Question 13

What is the characteristic thing seen on histology of pemphigus vulgaris?

Answer 13

“Row of Tombostones”, intraepidermal split blisters just above the dermis

Question 14

Who frequently diagnoses pemphigus vulgaris?

Answer 14

Dentists (LOL); commonly starts as mucosal erosions

Question 15

Describe the blisters seen in pemphigus vulgaris. Where are they located?

Answer 15

Flaccid and easily broken; commonly on the head, chest, and back

Question 16

Are there serum antibodies in pemphigus vulgaris?

Answer 16

Yes; their serum concentration (of desmoglein 1 & 3) can serve as marker for disease progression

Question 17

What is Nikolksy sign?

Answer 17

When the top layers of skin slip away from the underlying layers upon light pressure

+ in Pemphigus Vulgaris (…and Stevens Johnson Syndrome/TEN)

• in Bullous Pemphigoid (tense blister, doesnt sheer laterally under pressure)

Question 18

In Pemphigus vulgaris, where do you want to take the skin biopsy from?

Answer 18

The very outer edge of inflammation/erosion, adjacent to the normal skin

Question 19

What specific drug has show great efficacy in pemphis vulgaris?

Answer 19

Rituximab

Question 20

What is Dermatitis herpetiformis?

Answer 20

Autoimmune bullous disease linked to celiac’s and gluten hypersensitivity

Question 21

Dermatitis Herpetiformis: What type are the autoantibodies and what do they react with?

Answer 21

IgA; against tissue transglutaminase and cross react with epidermal transglutaminase

• Pt develops IgA antibodies to tissue transglutaminae (t-TG in gut)
• t-TG cross reacts with epidermal transglutaminase (e-TG in skin)

Question 22

Where is transglutaminase found in the skin?

Answer 22

Dermal capillaries and the basal cells of the epidermis

Question 23

Epidemiology of dermatitis herpetiformis

Answer 23

Men>women; onsets between 30-40

Question 24

Mucosal involvement in dermatitis herpetiformis?

Answer 24

NO; helps you distinguish between the other bullous diseases

Question 25

Describe the lesions in dermatitis herpetiformis. Where are they located?

Answer 25

Intensely itchy rash, possibly vesicular; commonly presents on the elbows, buttocks, knees, scalp, and neck

Question 26

Relation between dermatitis herpetiformis and gluten sensitive enteropathy

Answer 26

ALL those that have dermatitis herpetiformis also have gluten sensitive disease; not vice versa

Question 27

Histology of dermatitis herpetiformis

Answer 27

Lots of neutrophils, eosinophils infiltrating the dermal papillae

Question 28

Treatment of dermatitis herpetiformis

Answer 28

Strict adherence to gluten free diet; steroids are NOT helpful

Question 29

Dapsone used to treat which of these 3 diseases? Mechanism of action?

Answer 29

Treat Dermatitis herpetiformis. Prevents neutrophil infiltration into dermal papillae

Question 30

What disease associations does dermatitis herpetiformis have?

Answer 30

Non-hodgkin’s lymphoma (most be worked up); other autoimmune disorders, Thyroid disease, DM, connective tissue disease