First Aid, Chapter 8, Immunologic Disorders, Immunologic Skin Disease

Question 1

What are the target skin structures for pemphigus?

Answer 1

Desmogleins-1 and -3 in desmosome for pemphigus

Question 2

What are the target skin structures for pemphigoid and linear IgA bullous dermatosis?

Answer 2

BP Ags (BP180 and BP230) in hemidesmosome/lamina lucida for pemphigoid and linear IgA bullous dermatosis.

Question 3

What are the target skin structures for epidermolysis bullosa acquisita?

Answer 3

Type VII collagen or anchoring fibrils for epidermolysis bullosa acquisita

Question 4

What are the clinical signs of pemphigus?

Answer 4

Flaccid bullae on noninflamed skin Nikolsky’s sign Found on scalp, chest, oral mucosa, intertriginous areas

Question 5

What is the target of pemphigus vulgaris?

Answer 5

IgG epithelial surface Desmoglein-3 +/– desmosome

Question 6

What is the immunopathology of pemphigus vulgaris?

Answer 6

Epidermal IgG/C3

Question 7

What is the treatment of pemphigus vulgaris?

Answer 7

prednisone +- immunosuppresives

Question 8

What is the target of pemphigus folicaceous?

Answer 8

Desmoglein-1 +/– desmosome

Question 9

What is the immunopathology of pemphigus foliaceous?

Answer 9

Epidermal IgG/C3

Question 10

What is the treatment of pemphigus foliaceous?

Answer 10

Steroids

Question 11

What is the target of IgA pemphigus?

Answer 11

IgA epith surface Desmocollin-1

Question 12

What is the immunopathology of IgA pemphigus?

Answer 12

Epidermal IgA

Question 13

What is the treatment of IgA pemphigus?

Answer 13

Steroids

Question 14

What are the clinical findings of bullous pemphigoid?

Answer 14

Tense bullae on urticarial base
Pruritus
Flexural areas
Elderly

Question 15

What is the target of bullous pemphigoid?

Answer 15

IgG basement membrane zone (BMZ)
BP180
BP230

Question 16

What is the immunopathology of bullous pemphigoid?

Answer 16

IgG/C3 (BMZ)

Question 17

What is the treatment of bullous pemphigoid?

Answer 17

Steroids

Immuno-suppressives +/–

Question 18

What are the clinical signs of herpes gestationis?

Answer 18

Urticaria 
Blistering  
Periumbilical area  
During or immediately postpartum 
May flare w OCP/menses

Question 19

What is the target of herpes gestationis?

Answer 19

IgG BP180 BMZ

Question 20

What is the immunopathology of herpes gestationis?

Answer 20

Linear BMZ C3

Question 21

What is the treatment of herpes gestationis?

Answer 21

Steroids

Antihistamines

Question 22

What are the affected areas of epidermolysis bullosa acquisita?

Answer 22

Areas of trauma

Oral mucosa

Question 23

What is the target of epidermolysis bullosa acquisita?

Answer 23

Type VII collagen

Question 24

What is the immunopathology of epidermolysis bullosa acquisita?

Answer 24

Linear BMZ IgG/C3

Linear IgA/IgM

Question 25

What is the treatment of epidermolysis bullosa acquisita?

Answer 25

Colchicine or dapsone + steroids Immuno-suppressives +/ – for refractory cases

Question 26

What are the clinical signs of dermatitis herpetiformis?

Answer 26

Small grouped vesicles LE/buttocks Intense pruritus Associated with celiac disease

Question 27

What is the target of dermatitis herpetiformis?

Answer 27

Endomysial IgA Transglutaminase IgA Epidermal transglutaminase

Question 28

What is the immunopathology of dermatitis herpetiformis?

Answer 28

Granular BMZ IgA

Question 29

What is the treatment of dermatitis herpetiformis?

Answer 29

Eliminate gluten from diet

Question 30

What is the mnemonic for the key features of pemphigus?

Answer 30

DEsMO (Desmosome) = DEMO. DEMO is on the surface (of skin); so, when DEMO is affected, the surface of skin is damaged, leading to flaccid bullae.

Question 31

A 70-year-old woman presents with a pruritic rash over her upper extremities with what appear to be tense, fluid-filled blisters. What is the diagnosis?

Answer 31

Bullous pemphigoid. Tends to occur in the elderly, is pruritic, and occurs in flexural areas

Question 32

What does tissue immunofluorescence show for lupus erthematosis?

Answer 32

Granular immune deposits at BMZ in both involved and uninvolved skin (lupus band), IgM = C3 >IgG >IgA, where two or more required. Refer to Tables 8-25 and 8-29 for antigen testing in these conditions.