Tamai et al reported a severity index for evaluating a patient with junctional epidermolysis bullosa (JEB). The authors are from the Japanese Study Group for Rare Intractable Skin Diseases.
Parameters:
(1) de novo blister
(2) area of blistering (percent body surface area, BSA)
(3) oral blister
(4) height as percent of normal
(5) weight as percent of normal
(6) anemia (hemoglobin as percent of normal hemoglobin)
(7) serum protein as a percent of normal
(8) infection with methicillin resistant Staphylococcus aureus (MRSA)
|
Parameter |
Findings |
Points |
|
de novo blisters |
none |
0 |
|
|
1 to 3 per week |
1 |
|
|
>= 4 per week |
2 |
|
|
every day |
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 |
3 |
|
height |
100% |
0 |
|
|
91 - 99% |
1 |
|
|
80 - 90% |
2 |
|
|
< 80% |
3 |
|
weight |
100% |
0 |
|
|
91 - 99% |
1 |
|
|
80 - 90% |
2 |
|
|
< 80% |
3 |
|
anemia |
100% |
0 |
|
|
91 - 99% |
1 |
|
|
80 - 90% |
2 |
|
|
< 80% |
3 |
|
serum protein |
100% |
0 |
|
|
91 - 99% |
1 |
|
|
80 - 90% |
2 |
|
|
< 80% |
3 |
|
infection with MRSA |
none |
0 |
|
|
local, inactive infection |
1 |
|
|
local, active infection |
2 |
|
|
systemic |
3 |
total score =
= SUM(points for all 8 parameters)
Interpretation:
• minimum score: 0
• maximum score: 24
|
Total Score |
Disease Severity |
|
0 - 7 |
mild |
|
8 - 14 |
moderate |
|
15 - 24 |
severe |
A patient is classified as having severe disease if the score is >= 1 and one or more of the following:
(1) squamous cell carcinoma
(2) reactive amyloidosis
(3) defect in laminin 5
(4) family history of a lethal case
(5) pyloric atresia
Specialty: Dermatology
