Epidermolysis Bullosa

Question 1

Define the disorder?

Answer 1

Its a group of inherited disorders → that involve formation of blisters following trivial trauma →mechano bullous disorder

Question 2

Pathognomonic?

Answer 2

Vesicles & bulla within skin & mucous membranes

Question 3

Defect?

Answer 3

If Genetic → keratin & collagen VII gene defect
Rarely acquired→ immune mediated

Question 4

Classification?
Severity?

Answer 4

EB simplex → mild severity
EB dominant dystrophic → moderate
EB junctional
EB recessive dystrophic → severe involvement of skin, nail, mucosa

Question 5

Rx?

Answer 5

Prevention of trauma → by careful handling → to prevent blistering
Surgical treatment of deformities