Introduction
Methods
Assessments
Modified Localized Scleroderma Skin Severity Index
Cutaneous induration
Visual analogue scale
Punch biopsy
Epidermal atrophy
|
Papillary dermis fibrosis
| ||||
---|---|---|---|---|---|
Normal | 0 | More than seven layers | Normal | 0 | Absence |
Light | 1 | Six to seven layers | Light | 1 | Reduction in thickness of lax tissue |
Moderate | 2 | Four to five layers | Moderate | 2 | Segmental hyalinization |
Severe | 3 | Three layers or less | Severe | 3 | Complete hyalinization |
Dermal and subcutaneous infiltration
|
Reticular dermis fibrosis
| ||||
Normal | 0 | Absence | Normal | 0 | Absence |
Light | 1 | Isolated lymphocytes | Light | 1 | Small pockets of hyalinization |
Moderate | 2 | Small groups of lymphocytes | Moderate | 2 | Presence of moderate hyalinization |
Severe | 3 | Large groups of lymphocytes | Severe | 3 | Total hyalinization |
Adipose tissue fibrosis
|
Annex atrophy
| ||||
Normal | 0 | Absence | Normal | 0 | Absence |
Light | 1 | Light thickening of adipose septa | Light | 1 | Periadnexal fibrosis |
Moderate | 2 | Moderate thickening of adipose septa | Moderate | 2 | Reduction in size of annexes |
Severe | 3 | Severe thickening of adipose septa | Severe | 3 | Presence of adnexal vestiges |
Safety evaluation
Treatment
Statistical analysis
Results
Patient
|
Sex
|
Age (yr)
|
Time of evolution (yr)
|
Previous treatment
|
Type of scleroderma
|
Size of smallest lesion (cm
2
)
|
Size of largest lesion (cm
2
)
|
Affected segments
|
---|---|---|---|---|---|---|---|---|
1 | F | 47 | 10 | Yes | Linear | 10.5 | 97.5 | 1 |
2 | F | 24 | 1.5 | No | Linear | 1.5 | 17.5 | 1 |
3 | F | 77 | 3 | Yes | Generalized | 1 | 412.5 | 6 |
4 | F | 66 | 6 | Yes | Generalized | 2 | 255 | 5 |
5 | F | 54 | 3 | Yes | Generalized | 31.5 | 448 | 3 |
6 | F | 29 | 3 | Yes | Circumscribed | 360 | 765 | 2 |
7 | F | 21 | 10 | Yes | Circumscribed | 45 | 52.2 | 1 |
8 | M | 31 | 8 | No | Circumscribed | 0.48 | 7 | 1 |
9 | M | 39 | 3 | Yes | Generalized | 0.25 | 136 | 13 |
10 | F | 46 | 2 | Yes | Circumscribed | 12 | 22.5 | 1 |
11 | M | 51 | <1 | No | Generalized | 1 | 36 | 3 |
12 | F | 48 | 4 | No | Generalized | 6 | 189 | 2 |
Mean | NA | 46.5 | 3 | NA | NA | 4 | 116.75 | 2 |
Maximum | NA | 77 | 10 | NA | NA | 360 | 765 | 13 |
Minimum | NA | 21 | 1.5 | NA | NA | 0.25 | 7 | 1 |
Patient
|
Initial mLoSSI
|
Final mLoSSI
|
Improvement mLoSSI (%)
|
Mean basal durometer
|
Mean final durometer
|
Improvement durometer (%)
|
Initial VAS hardness
|
Final VAS hardness
|
Improvement VAS hardness (%)
|
Initial VAS pruritus
|
Final VAS pruritus
|
Improvement VAS pruritus (%)
|
---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 1 | 0 | 100 | 56.73 | 49.12 | 13.41 | 0 | 4 | −4 | 0 | 5 | −5 |
2 | 3 | 0 | 100 | 35.6 | 34.51 | 3.06 | 0 | 0 | 0 | 0 | 0 | 0 |
3 | 14 | 6 | 57 | 36.95 | 38.09 | −3.08 | 7 | 5 | 29 | 0 | 0 | 0 |
4 | 11 | 1 | 93 | 28.37 | 30.27 | −6.7 | 8 | 2 | 75 | 7 | 2.5 | 65 |
5 | 9 | 0 | 100 | 33.71 | 31.14 | 7.62 | 5 | 3 | 40 | 8 | 3 | 62 |
6 | 3 | 0 | 100 | 26.21 | 27.03 | −3.13 | 6.5 | 1.5 | 77 | 6.5 | 2 | 69 |
7 | 2 | 0 | 100 | 32.49 | 35.39 | −8.93 | 10 | 4.5 | 55 | 5 | 2.5 | 50 |
8 | 2 | 1 | 50 | 30.23 | 26.26 | 13.13 | 5 | 3 | 40 | 0 | 0 | 0 |
9 | 4 | 2 | 50 | 45.52 | 42.02 | 7.69 | 7 | 3 | 57 | 5 | 2 | 60 |
10 | 3 | 0 | 100 | 42.68 | 35.29 | 17.31 | 8 | 0 | 100 | 5 | 0 | 100 |
11 | 14 | 0 | 100 | 36.83 | 27.05 | 26.56 | 0 | 0 | 0 | 3 | 0 | 100 |
12 | 4 | 0 | 100 | 24.18 | 13.51 | 44.12 | 3 | 3 | 0 | 8 | 0 | 100 |
Mean | 5.83 | 0.83 | NA | 35.79 | 32.47 | NA | 4.96 | 2.42 | NA | 3.96 | 1.42 | NA |
P-valueb
| 0.002 | 0.05 | 0.032 | 0.065 |
Epidermal atrophy
|
Dermal infiltration
|
Adipose tissue infiltration
|
Papillary dermis fibrosis
|
Reticular dermis fibrosis
|
Adipose tissue fibrosis
|
Adnexal atrophy
| ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Basal
|
Final
|
Basal
|
Final
|
Basal
|
Final
|
Basal
|
Final
|
Basal
|
Final
|
Basal
b
|
Final
c
|
Basal
|
Final
| |
Absence | 1 | 4 | 0 | 0 | 4 | 3 | 1 | 3 | 1 | 4 | 2 | 0 | 1 | 1 |
Light | 1 | 4 | 4 | 8 | 3 | 9 | 6 | 3 | 4 | 2 | 3 | 3 | 4 | 3 |
Moderate | 4 | 1 | 5 | 3 | 5 | 0 | 2 | 3 | 1 | 6 | 2 | 1 | 3 | 3 |
Severe | 6 | 3 | 3 | 1 | 0 | 0 | 3 | 3 | 6 | 0 | 4 | 5 | 4 | 5 |
Mean (SD) | 2.25 (0.96) | 1.25 (1.21) | 1.92 (0.79) | 1.42 (0.67) | 1.08 (0.90) | 0.75 (0.45) | 1.58 (1.0) | 1.5 (1.17) | 2 (1.13) | 1.17 (0.94) | 1.72 (1.19) | 2.22 (0.97) | 1.83 (1.03) | 2 (1.04) |
P-valued
| 0.010 | 0.034 | 0.305 | 0.705 | 0.039 | 0.276 | 0.581 |