Pemphigus Vulgaris

Question 1

In PV, sometimes the esophagus is involved. What is it called when there’s sloughing of the entire lining of the esophagus, forming a cast?

Answer 1

Esophagitis dessicans superficialis

Question 2

True or false: in unaffected family members of patients with PV, specific antibodies for PV may be present.

Answer 2

True; this is true of all autoimmune disorders

Question 3

1) What are the two possible antibodies that may cause PV?
2) Which antibody, or antibodies, must be present if there’s mucosal involvement?

Answer 3

1) Desmoglein 1 and 3
2) Desmoglein 1 and 3 must both be present if there’s both cutaneous and mucosal involvment. Patients with ONLY mucosal involvment only have antibodies to desmoglein 3.

Question 4

True or false: PV can be associated with myasthenia gravis and thymoma.

Answer 4

True

Question 5

What are the direct immunofluorescence finding associated with PV?

Answer 5

A “chicken-wire” pattern of intercellular IgG and/or C3 in perilesional skin or plucked hairs

Question 6

Where is the level of the acantholysis seen histologically in patients with PV?

Answer 6

In the suprabasilar layer of the epidermis. Classically there is “tombstonning” of the basal keratinocytes beneath the intraepidermal blister.