57 - Intercellular Immunoglobulin A Dermatosis (IgA Pemphigus)

Question 1

Major types of intercellular immunoglobulin A dermatosis

Answer 1

1. Subcorneal pustular dermatosis (SPD)-type IAD

2. Intraepidermal neutrophilic dermatosis (IEN)-type IAD

Question 2

Superficial flaccid pustules in the periphery of annular or herpetiform erythemas most prevalently on the intertriginous sites

Answer 2

Subcorneal pustular dermatosis-type IAD

Question 3

Atypical pustular skin lesions with a sunflower-like configuration

Answer 3

Intraepidermal neutrophilic dermatosis-type IAD

Question 4

Autoantigen in SPD-type IAD

Answer 4

Desmocollin 1

Question 5

Autoantigen in IEN-type IAD

Answer 5

Unknown; probably a nondesmosomal protein

Question 6

Desmocollin _____ is expressed in the keratinocyte cell surfaces in the uppermost epidermis

Answer 6

1

Question 7

Desmocollin _____ is expressed in the entire epidermis

Answer 7

2

Question 8

Desmocollin _____ is expressed more strongly in the low epidermis

Answer 8

3

Question 9

SPD vs IEN-type IAD: develops oral mucosal lesions

Answer 9

IEN-type

Question 10

IIF substrate for IAD

Answer 10

Normal human skin or monkey esophagus

Question 11

Prognosis: IAD vs classical IgG pemphigus

Answer 11

IAD has a much better prognosis and is rarely fatal

Question 12

Most common therapy in IAD

Answer 12

Oral dapsone 50-200 mg/day

Question 13

Second choice of therapy for IAD

Answer 13

Systemic corticosteroids