Hashimoto reported a severity index for neonates with epidermolysis bullosa.
Parameters:
(1) de novo blister
(2) area of blistering (percent body surface area, BSA)
(3) oral blister
(4) feeding difficulty
(5) weight change
(6) infection with methicillin resistant Staphylococcus aureus (MRSA)
|
Parameter |
Findings |
Points |
|
de novo blisters |
none |
0 |
|
|
1 to 4 per week |
1 |
|
|
5 to 10 per week |
2 |
|
|
> 10 per week |
3 |
|
area of blistering |
none |
0 |
|
|
1 - 4% |
1 |
|
|
5 - 15% |
2 |
|
|
> 15% |
3 |
|
oral blister |
none |
0 |
|
|
1 or 2 per month |
1 |
|
|
several per month |
2 |
|
|
persistent, always |
3 |
|
feeding difficulty |
none |
0 |
|
|
mild |
1 |
|
|
intermittent |
2 |
|
|
severe |
3 |
|
weight change |
normal increase |
0 |
|
|
slight increase |
1 |
|
|
no change |
2 |
|
|
loss |
3 |
|
infection with MRSA |
none |
0 |
|
|
local, inactive infection |
1 |
|
|
local, active infection |
2 |
|
|
systemic |
3 |
total score =
= SUM(points for all 6 parameters)
Interpretation:
• minimum score: 0
• maximum score: 18
|
Total Score |
Disease Severity |
|
0 - 5 |
mild |
|
6 - 10 |
moderate |
|
11 - 16 |
severe |
A patient is classified as having severe disease if the score is >= 1 and one or more of the following:
(1) defect in laminin 5
(2) pyloric atresia
(3) family history of lethal epidermolysis bullosa
(4) family history of severe recessive dystrophic epidermolysis bullosa
(5) family history of epidermolysis bullosa with muscular dystrophy
Specialty: Dermatology
