Pemphigus

Question 1

Name the subtypes of Pemphigus

Answer 1

P. vulgaris, P. foliaceus, paraneoplastic P., IgA P.

Question 2

Incidence and onset age

Answer 2

2/1 Mio./Year
50-60 years
Prevalence 5/10.000 in Ger

Question 3

Against which molecules are the Auto-ab in pemphigus directed? Where is the split-formation?

Answer 3

Split formation: intraepidermal

P. vulgaris: Desmoglein 3 (lower layers of epidermis), whe skin is affected also against Dsm1

P. foliaceus: Desmoglein 1 (upper layers of epidermis)

Question 4

Whats the Nikolski-Phenomenon?

Answer 4

Slight rubbing of the skin leads to blistering (in the gluteal region, this may lead to infections!)

Question 5

What is acantholysis?

Answer 5

Loss of intercellular connections (desmosomes),
loss of cohesion of keratinocytes,
in P. vulgaris (not in bullous pehmphigoid!)

Question 6

What is the tandem peptide?

Answer 6

Favours oligomerization with Dsg3 -> inhibits p38MAPK activation -> prevents disease in pemphigus mice

Question 7

What kind of pemphigus mouse models exist?

Answer 7

Active (lymphocyte transfer) Lymphocytes from immunized Dsg3-/- mice into Rag-/-

Passive (ab-transfer) anti-Dsg3 AK1489 ab

Question 8

What is immunoadsorption-plasmapheresis?

Answer 8

Adsorption of AB (or DSG-specific ab) from the patients blood
Good results in combination with prednisolone

Question 9

Whats another auto-ab in Pemphigus?

Answer 9

Desmocollin Auto-ab -> skin blistering in mice (anti-Dsc)

Dsc3 binding is crucial for keratinocyte cohesion

rare in p.

Question 10

Which biological is used for Pemphigus?

Answer 10

Rituximab (aCD20 ab)