Tamai et al reported a severity index for evaluating a patient with dystrophic epidermolysis bullosa (DEB). 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) scar formation
(5) pseudosyndactyly
(6) esophageal stenosis
(7) height as percent of normal
(8) weight as percent of normal
(9) anemia (hemoglobin as percent of normal hemoglobin)
(10) serum protein as a percent of normal
(11) bone minerals as percent of normal
(12) 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 |
|
scar formation |
none |
0 |
|
|
mild |
1 |
|
|
intermittent |
2 |
|
|
severe |
3 |
|
pseudosyndactyly |
none |
0 |
|
|
mild |
1 |
|
|
intermittent |
2 |
|
|
severe |
3 |
|
esophageal stenosis |
none |
0 |
|
|
dysphagia |
1 |
|
|
unable to eat solid material |
2 |
|
|
unable to take liquids |
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 |
|
bone minerals |
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 12 parameters)
Interpretation:
• minimum score: 0
• maximum score: 36
|
Total Score |
Disease Severity |
|---|---|
|
0 - 10 |
mild |
|
11 - 18 |
moderate |
|
19 - 26 |
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
Specialty: Dermatology
