Table 4.
Characteristic | Suggestive-or-compatible challenge | Controls | p | ||
---|---|---|---|---|---|
n = 17 | Missing | n = 121 | Missing | ||
Age, mean/median (range), years | 69/71 (48–81) | 66.4/66 (38–96) | 0.46 | ||
Weight, mean/median (range), kg | 87/80 (55–154) | 72.6/73 (44–114) | 0.02 | ||
BMI, mean/median (range), kg/m2 | 32/29 (24–45) | 26.1/25 (18–40) | 0.01 | ||
Female/male, n (%); sex ratio | 9 (53%)/8 (47%); 1.1 | 0 | 69 (57%)/52 (43%); 1.3 | 0 | |
Involved sites, mean/median (range), n | 2.8/3 (2–5) | 2.2/2 (1–5) | |||
MMP involvement, n (%) | 0 | 2 | |||
Isolated MM | 3 (18%) | 59 (50%) | |||
MM and cutaneous | 14 (82%) | 60 (50%) | |||
DIF, yes/no, n (%) | |||||
IgA deposits | 0 | 0 | 26 (22%)/94 (78%) | 1 | 0.04 |
IgG deposits | 14 (82%)/3 (18%) | 0 | 89 (74%)/31 (26%) | 1 | |
C3 deposits | 14 (82%)/3 (18%) | 0 | 85 (71%)/35 (29%) | 1 | |
Direct IEM, n (%) | |||||
LD ± LL | 7 (87%) | 9 | 62 (60%) | 18 | |
Upper LL ± HD | 1 (13%) | 13 (13%) | |||
IIF on SSS, n (%) | |||||
Roof | 4 (27%) | 2 | 22 (21%) | 18 | |
Floor | 0 | 2 (2%) | |||
Mixed | 0 | 3 (3%) | |||
Negative | 11 (73%) | 76 (74%) | |||
ELISA, positive/negative, n (%) | |||||
BP230 | 3 (20%)/13 (80%) | 2 | 10 (13%)/68 (87%) | 43 | |
BP180 | 7 (47%)/8 (53%) | 2 | 43 (51%)/41 (49%) | 37 | |
Involvement, yes/no, n (%) | |||||
Cutaneous | 14 (82%)/3 (18%) | 60 (50%)/59 (50%) | 0.02 | ||
Buccal | 11 (65%)/6 (35%) | 0 | 101 (89%)/12 (11%) | 8 | 0.01 |
Laryngeal | 6 (35%)/11 (65%) | 0 | 34 (30%)/79 (70%) | 8 | |
Genital and/or anal | 8 (47%)/9 (53%) | 0 | 35 (31%)/78 (69%) | 8 | |
Conjunctival | 2 (12%)/15 (88%) | 0 | 28 (25%)/85 (75%) | 8 | 0.36 |
Esophageal | 0 | 0 | 6 (5%)/107 (95%) | 8 | |
Severe | 7 (41%)/10 (59%) | 0 | 78 (67%)/39 (33%) | 8 | 0.05 |
At 1 year of follow-up, yes/no, n (%) | |||||
Complete remission | 9 (82%)/2 (18%) | 6 | 68 (56%)/53 (44%) | 0 | 0.12 |
Relapses/flares | 4 (33%)/8 (67%) | 5 | 28 (23%)/93 (77%) | 0 | |
Deaths | 0/17 (100%) | 0 | 2 (2%)/119 (98%) | 0 |
MMP, mucous membrane pemphigoid; BMI, body mass index; MM, mucous membrane; DIF, direct immunofluorescence; Ig, immunoglobulin; IEM, immunoelectron microscopy; LD, lamina densa; LL, lamina lucida; ±, with or without; HD, hemidesmosome; IIF, indirect immunofluorescence; SSS, salt-split skin; ELISA, enzyme-linked immunosorbent assay.