Dill et al reviewed the Index for Facial Angiofibromas (IFA) which can be used to evaluate a patient with facial angiofibromas. The presence of facial angiofibromas is associated with the tuberous sclerosis complex. The authors are from multiple institutions in Europe, the United States and Japan.
Patient selection: facial angiofibromas
Parameters:
(1) erythema
(2) redness of facial angiofibromas (FA)
(3) extent of red facial angiofibroma in relation to all affected areas
(4) diameter of largest facial angiofibroma in mm
(5) alar facial groove affected
(6) extent of all facial angiofibromas on nose
(7) extent of all facial angiofibromas on cheeks
(8) extent of all facial angiofibromas on chin
Parameter
Finding
Points
erythema
none
0
light red
1
marked red
2
redness of FA
none
0
light red
1
marked red
2
extent of red FA vs all FA
none
0
sporadic
1
less than 50%
2
more than 50%
3
diameter of largest FA
none
0
< 3 mm
1
3 to 10 mm
2
> 10 mm
3
alar facial groove
no
0
yes
1
percent FA on nose
0
0
< 50%
1
> 50%
2
cluster
3
percent FA on cheeks
0
0
< 50%
1
> 50%
2
cluster
3
percent FA on chin
0
0
< 50%
1
> 50%
2
cluster
3
where:
• A cluster is a high density of angiofibromas without space between.
total score =
= SUM(points for all parameters)
Interpretation:
• minimum score: 0
• maximum score: 20
• A change in the IFA is expected in a patient responding to mTOR inhbitors.
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