mucous membrane pemphigoid (Cicatricial pemphigoid)

Question 1

Patient age for mucous membrane pemphigoid?

Answer 1

• elderly 60-80

Question 2

Pathogenesis of Mucous membrane pemphigoid:

Answer 2

• autoreactive IgG antibodies directed against various antigens in anchoring filament zone (vs hemidesmosomal plaque proteins in conventional BP)

Question 3

Three subgroups of Mucous membrane pemphigoid are:

Answer 3

1. Anti-epiligrin MMP
2. Ocular MMP
3. Anti-BP antigen MMP

Question 4

Anti-epiligrin MMP subtype of Mucous membrane pemphigoid targets ______

Answer 4

• laminin 332

Question 5

Anti-epiligrin MMP subtype of Mucous membrane pemphigoid salt-split skin will show_____

Answer 5

• dermal staining

Question 6

Anti-epiligrin MMP subtype of Mucous membrane pemphigoid is a/w underlying ________, most commonly _______

Answer 6

- solid organ malignancy

• adenocarcinoma

Question 7

Ocular MMP subtype of Mucous membrane pemphigoid targets _____

Answer 7

• Beta 4 subunit of alpha 6 beta 4 integrin

Question 8

Ocular MMP subtype of Mucous membrane pemphigoid affects which regions of body?

Answer 8

• nearly exclusive ocular involvement

Question 9

Anti-BP antigen MMP subtype of mucous membrane pemphigoid targets ______

Answer 9

• BP180 (C-terminus)

Question 10

Mucous membrane pemphigoid can commonly cause ______ at sites of involvement.

Answer 10

• scarring

Question 11

What are some of the sequelae of Mucous membrane pemphigoid when involving the conjunctiva?

Answer 11

• symblepharon (adhesion of bulbar and palpebral conjunctivae)
• trichiasis (inward facing eyelashes)
• ectropion or entropion

- blindness

Question 12

Most common site involved in Mucous membrane pemphigoid:

Answer 12

• oral mucosa (causing erosions of gingiva (desquamative gingivitis)

Question 13

If there is skin involvement in Mucous membrane pemphigoid, what is a clinical factor that can help differentiate from BP?

Answer 13

• lesions will heal with atrophic scars (not seen in BP)

Question 14

Describe the Brunsting-perry variant of Mucous membrane pemphigoid?

Answer 14

• lesions localized to head and neck—> scarring alopecia
• no mucosal involvement

Question 15

Histology of Mucous membrane pemphigoid:

Answer 15

• similar to BP with fewer eos and increased dermal fibrosis/scarring

Question 16

DIF of Mucous membrane pemphigoid will show:

Answer 16

• IgG, IgA, and/or C3 along BMZ

Question 17

Salt-split skin of Mucous membrane pemphigoid will show what for each of the three subtypes:

Answer 17

• epidermal roof staining for all subtypes except anti-epiligrin MMP (will have dermal/floor staining)

Question 18

First line treatment for Mucous membrane pemphigoid?

Answer 18

• Dapsone

Question 19

Treatment for severe or progressive ocular involvement of Mucous membrane pemphigoid:

Answer 19

• cyclophosphamide + steroids or steroid sparing immunossuppressive (MMF/azathioprine)