Clinical: Bullous Disease

Question 1

Pathogenesis of the Immunobullous diseases.

Answer 1

Autoantibodies against skin proteins.

Question 2

Pathogenesis of Pemphigus Vulgaris (PV)

Answer 2

Antibodies mainly against Desmoglein 3 (interkeratinocyte protein)

Question 3

Pathogenesis of Pemphigus Foliaceous (PF)

Answer 3

Antibodies against Desmoglein 1 (interkeratinocyte protein)

Question 4

Differentiating factor of P. Vulgaris vs. Foliaceous on PE

Answer 4

Vulgaris has oral involvement
Foliaceous does not
(also foliaceous looks more eroded thru the skin)

Question 5

Modality to diagnose Pemphigus diseases.

Answer 5

H&E stain of epidermis
PV: lesional gap between the stratum basalis and the other epidermal layers
PF: stratum corneum shows a focal spot of thinning (erosion)

Question 6

First line treatment for PV and PF.

Answer 6

Systemic Corticosteroids

Question 7

Trial monoclonal Ab drug used for PV and PF.

Answer 7

Rituximab

MOA: anti-CD20 antibody

Question 8

Pemphigus condition associated with Non-Hodgkins lymphoma.

Answer 8

Paraneoplastic Pemphigus

Question 9

Pathogenesis of Pemphigoid Diseases

Answer 9

Auto Abs against proteins in the dermoepidermal junction

Question 10

Pathogenesis of Bullous Pemphigoid

Answer 10

Abs against Bullous Pemphigoid Antigen 1 and 2

Question 11

How are pemphigoid lesions differentiated from pemphigus lesions?

Answer 11

Pemphigus lesions are more flaccid bullae that rupture and because they are more superficial, less scarring occurs.

Pemphigoid lesions are more tense bullae that don’t rupture as often but usually scar. Pemphigoid looks much worse than it is and usually can go into remission without treatment

Question 12

First line treatment for Bullous Pemphigoid

Answer 12

Systemic Corticosteroids

Question 13

Pathogenesis of Mucous Membrane Pemphigoid

Answer 13

Antibodies against:
-beta 4 integrin
-alpha 6 integrin
-BPAg2 (Bullous Pemphigus Antigen)
-Laminin 332
(eye and esophageal ulcers are common)

Question 14

Treatment for the two types of Mucous Membrane Pemphigoid

Answer 14

Non-scarring: topical corticosteroids, anti-inflammatory meds

Scarring: systemic corticosteroids/cyclophosphamide combo

Question 15

Serous sequelae of Mucous Membrane Pemphigoid

Answer 15

Blindness
Esophageal stenosis
Laryngeal Stensosis

Question 16

Pathogenesis of Epidermolysis Bullosa Acquisita

Answer 16

Antibodies against Type VII collagen in the papillary dermal layer.
Forms bullae on acral skin ( finger tips, knuckles, elbows, knees, buttocks, toes, heels, ears)

Question 17

Treatment for Epidermolysis Bullosa Acquisita

Answer 17

Systemic Corticosteroids, cyclosporine, colchicine, rituximab

Question 18

Pathogensis of Linear IgA disease

Answer 18

IgA antibodies directed against a malformed skin protein (usually truncated BPAg2)

Question 19

Most common trigger for Linear IgA disease

Answer 19

Vancomycin use

Question 20

Treatment for Linear IgA disease

Answer 20

Stop using vanco

Dapsone

Question 21

Pathogenesis of Dermatitis Herpetiformis

Answer 21

Skin manifestation of Celiac Sprue (gluten sensitive enteropathy)

Question 22

Most common location of vesicle lesions of Dermatitis Herpetiformis

Answer 22

Extensor surfaces

Question 23

First line treatment for Dermatitis Herpetiformis

Answer 23

Initiate Dapsone with a gluten free diet

Question 24

Pathogenesis of Porphyria Cutanea Tarda

Answer 24

Defect in uroporphyrinogen decarboxylase

-blisters appear on the dorsum of the hands