Lupus pernio is a chronic facial lesion due to sarcoidosis with a minimal rate of spontaneous remission. In order to quantitate response to therapy for lupus pernio, we developed a specific lupus pernio activity and severity index (LuPASI) based on the psoriasis activity and severity index.
We evaluated 10 patients with lupus pernio at one institution, were seen at least on two separate visits, and evaluated by two investigators (RPB and EEL) when possible. The face was divided into specific areas and each area was separately scored on a five point scale for erythema (E), induration (I), and desquamation (D). The total amount of the area (A) involved was also assessed on a 7 point scale. The divisions were the four quadrants of the face, with the division of upper and lower being through the mid eye, with the nose should be scored separately.
All 10 patients had one or more areas of involvement. There were 297 comparisons between the areas involved. The table below shows the difference between the median determination for each area and the discrepancy for the individual score for different days for the same patient. Level of Difference: None: E=76.4%,I=74.4%,D=77.1%, A=83.8%, All=77.9%; One: E=20.2%, I=23.2%, D=20.9%, A=16.2%, All=20.1%; Two: E=3.4%, I=2.0%; D=1.3%, A=0.0%, All=1.7%. Three: I=0.3%, All=0.1%. For all four descriptors, the agreement was with one point of the median in over 95% of cases. When we compared two observers (RPB vs EEL), there was less agreement, but in less than 5% of cases in which there was a 3 point or higher difference noted between the readers. The LuPASI score was then used at another institution. It was judged to simple to understand and easily completed in less than two minutes.
We conclude that the use of a LuPASI score can provide objective evidence of response to therapy.
When the same reader evaluates the patient, a greater than 1 point difference probably reflects response to therapy, rather than chance.
R.P. Baughman, Centocor