Bullous diseases

Question 1

Dermatitis Herpetiformis (DH) definition ?

Answer 1

Intensely itchy, chronic papulovesicular eruption distributed symmetrically
on extensor surfaces.

Question 2

Typical for pemphigus foliacus in direct immunoflurescence ?

Answer 2

Presence of IgG antibodies anti-desmglein 1

Question 3

Diagnosis of Dermatitis Herpetiformis ?

Answer 3

• Skin biopsy ; collections of neutrophils (microabscesses).

- DIF ; Granular IgA deposits in normal-appearing skin are diagnostic for DH.

Question 4

The gold standard of diagnosis of autoimmune bulllous diseases ?

Answer 4

Direct immunofluorescence

Question 5

Common complication of Pemphigus vulgaris ?

Answer 5

• secondary infection.

- disturbances of fluid and electrocyte.

Question 6

In which bullous disease we can see Acantholysis ? and explain it ?

Answer 6

Is the loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes (in other words the keratinocytes detach from their neighbours and float free in the blisters).
- Seen in diseases such as pemphigus vulgaris.

Question 7

Typical for dermatitis herpetiformis in direct immunoflurescence ?

Answer 7

Granular IgA in the dermal papillae. (even in normal-appearing skin)

Question 8

Paraneaoplastic pemphigus (PNP) combines features of what diseases?

Answer 8

• PNP lesions combine features of Pemighus V and erythema multiforme, clinically and histologically.
• mucus membrane primarily and most severely involved.
• associated with internal malignancy.

Question 9

Typical for pemphigoid in direct immunoflurescence ?

Answer 9

IgG at the basement membrane zone (lamina lucida). and also C3.

• IgG binds to two main antigens, most commonly to BP230 and BP180

Question 10

What is may be required in case of blisters in children ?

Answer 10

Electron microscopy.

Question 11

Most, if not all, DH patients have an associated with which condition ?

Answer 11

gluten-sensitive enteropathy

Question 12

Which Drugs can induce PV ?

Answer 12

most significantly in association
with PV are;

• Penicillamine
• captopril

Question 13

The biopsy for DIF in vesiculo-bullous diseases should be taken from ?

Answer 13

Peri-lesional skin.

Question 14

PEMPHIGUS ?

Answer 14

A serious, acute or chronic, bullous autoimmune disease of SKIN and MUCOUS MEMBRANES based on acantholysis.
- Common in the middle age groups(40-60 years
of life).

Question 15

What is the 1st line treatment for dermatitis herpetiformis ?

Answer 15

• Dapsone.

- 2nd -> glucocoticosteriods.

Question 16

Where does the pemphigus starts for most of the patients ?

Answer 16

• Oral mucosa.