Bullous pemphigoid

L12_PEMPHIGOID_BULL

bullous pemphigoid: Bullous pemphigoid (BP) is the most common form of autoimmune bullous dermatosis.

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Endpoint definition

Endpoint definition steps

FinnGen

Phenotype data

520210

1. Apply sex-specific rule

None

520210

2. Check conditions

None

520210

3. Check pre-conditions, main-only, mode, registry filters

Registry filters:

  • Hospital discharge: ICD-10 L12.0
  • Cause of death: ICD-10 L12.0

2 out of 7 registries used, show all original rules.

714

4. Check minimum number of events

None

714

5. Include endpoints

None

714

6. Filter based on genotype QC (FinnGen only)

714

Control definitions (FinnGen only)

Control exclude
L12_BULLOUS

Extra metadata

Level in the ICD hierarchy
4
First used in FinnGen datafreeze
DF2
Parent code in ICD-10
L12
Name in latin
Pemphigoides bullosus

Case counts by codes

FinnGen case counts by registry codes:

generic upset plot

Upset plot

Full data table

Summary Statistics

-FinRegistry-

Key figures

All Female Male
Number of individuals
Whole population 6427 3635 2742
Only index persons 5536 3158 2378
Unadjusted period prevalence (%)
Whole population 0.09 0.10 0.08
Only index persons 0.11 0.12 0.09
Median age at first event (years)
Whole population 78.57 79.60 77.21
Only index persons 78.21 79.15 76.97

-FinnGen-

Key figures

All Female Male
Number of individuals 714 340 374
Unadjusted period prevalence (%) 0.14 0.12 0.16
Median age at first event (years) 74.20 73.09 75.22

-FinRegistry-

Age distribution of first events

-FinnGen-

Age distribution of first events

-FinRegistry-

Year distribution of first events

-FinnGen-

Year distribution of first events

-FinRegistry-

Cumulative Incidence Function

-FinnGen-

Cumulative Incidence Function

CodeWAS (R11)

CodeWAS is a tool for exploring the associations between an endpoint and all of the medical codes and drug codes.

This is a new tool, please reach out using the contact form for feedback and improvement ideas.

First, a cohort is built by matching controls to the endpoint cases using year of birth and sex. Then, a Fisher test is done for all the medical codes and drug codes between the cases and controls of this cohort. Codes are reported in the table below if they have −log10(p-value) ≥ 6.

Matched cohort

Matched cases
626
Matched controls
6260
Code
Vocabulary
Description
Odds Ratio
-log10(p)
N matched cases
N matched controls
L12.0
ICD-10 Finland
Bullous pemphigoid
+∞
323.0
626
*
TQX10
NOMESCO Finland
Biopsy of skin and subcutaneous tissue
18.5
188.6
328
352
D07AD01
ATC
clobetasol; topical
11.5
142.8
318
517
D07AC01
ATC
betamethasone; topical
6.1
96.6
403
1425
L12.9
ICD-10 Finland
Pemphigoid, unspecified
1060.5
95.6
91
*
H02AB06
ATC
prednisolone; systemic
6.5
95.1
489
2217
L30.9
ICD-10 Finland
Dermatitis, unspecified
6.5
78.7
247
573
E0000UV
ATC
NA
4.8
74.5
390
1592
L04AX01
ATC
azathioprine; systemic
12.5
54.7
102
96
TQW80
NOMESCO Finland
Local dermatologic skincare
15.1
51.7
88
67
A12AX
ATC
Calcium, combinations with vitamin D and/or other drugs
3.6
49.4
351
1645
L04AX03
ATC
methotrexate; systemic (immunosuppressants)
6.4
47.9
138
265
135
Kela drug reimbursment
Pemphigus
+∞
44.3
42
*
L13.9
ICD-10 Finland
Bullous disorder, unspecified
82.7
41.5
46
6
D07AC13
ATC
mometasone; topical
3.4
40.3
266
1124
J04BA02
ATC
dapsone; oral
69.1
39.6
45
7
L12
ICD-10 Finland
Pemphigoid
92.2
39.3
43
5
L12.8
ICD-10 Finland
Other pemphigoid
+∞
37.9
36
*
D07BB04
ATC
hydrocortisone butyrate and antiseptics; topical
4.5
36.6
148
407
L12.1
ICD-10 Finland
Cicatricial pemphigoid
+∞
33.6
32
*
D07AB08
ATC
desonide; topical
4.3
30.0
122
332
E000001
ATC
NA
3.2
29.5
189
746
D07BC01
ATC
betamethasone and antiseptics; topical
3.5
25.8
134
448
SPAT1148
SPAT
Treatment of chronic ulcer or burn
3.0
24.6
169
690
D07AB02
ATC
hydrocortisone butyrate; topical
2.5
22.5
222
1114
L29.9
ICD-10 Finland
Pruritus, unspecified
8.7
20.8
45
55
ECA20
NOMESCO Finland
Biopsy of gingiva
19.1
20.6
31
17
XQ848
NOMESCO Finland
Dermatological photography
6.8
20.4
52
82
J01DB01
ATC
cefalexin; oral
2.5
19.4
515
4059
JN3AE
NOMESCO Finland
Abdominal ultrasound examination
3.0
19.4
127
492
R06AE07
ATC
cetirizine; oral
2.2
18.0
273
1646
A02BC02
ATC
pantoprazole; systemic
2.1
17.0
440
3296
D02AX
ATC
Other emollients and protectives
5.7
15.6
45
84
L13.0
ICD-10 Finland
Dermatitis herpetiformis
163.9
15.5
16
*
A46
ICD-10 Finland
Erysipelas
2.5
15.2
136
629
S97
ICPC
Chronic ulcer skin
3.4
15.0
75
239
A10AB05
ATC
insulin aspart; parenteral (insulins and analog. for injection, fast-acting)
3.3
14.9
78
257
A10AE04
ATC
insulin glargine; parenteral
3.0
14.7
91
335
GD1AA
NOMESCO Finland
Thorax X-ray examination
2.0
14.7
321
2188
S18
ICPC
Laceration/cut
2.0
14.6
303
2025
L97
ICD-10 Finland
Ulcer of lower limb, not elsewhere classified
4.2
14.2
54
136
L30.0
ICD-10 Finland
Nummular dermatitis
5.3
14.1
43
86
GD1QA
NOMESCO Finland
Supine thorax X-ray examination
2.3
14.1
158
814
S29
ICPC
Skin symptom/complaint other
2.4
13.8
136
659
S99
ICPC
Skin disease other
6.3
13.4
35
58
A10BH05
ATC
linagliptin; oral
3.4
13.3
65
204
SPAT1156
SPAT
Guidance concerning treatment of rash and skin
2.3
13.2
133
650
215
Kela drug reimbursment
Diabetes, non-insulin-treated
2.1
13.2
188
1072
L29.8
ICD-10 Finland
Other pruritus
10.8
12.8
24
23
L30.3
ICD-10 Finland
Infective dermatitis
5.2
12.7
39
79
C03CA01
ATC
furosemide; systemic
1.9
12.4
292
2003
D09AB01
ATC
zinc bandage without supplements; topical
6.3
12.3
32
53
E11.8
ICD-10 Finland
Type 2 diabetes mellitus, with unspecified complications
2.5
11.9
96
418
H02AB07
ATC
prednisone; oral
2.3
11.8
119
577
D02AE01
ATC
carbamide; topical
3.3
11.7
59
190
R21
ICD-10 Finland
Rash and other nonspecific skin eruption
4.1
11.5
44
112
NK6QA
NOMESCO Finland
Measurement of bone density from tow or more locations with X-ray
3.3
11.4
58
188
J45.9
ICD-10 Finland
Asthma, unspecified
2.2
11.1
133
696
E11.9
ICD-10 Finland
Type 2 diabetes mellitus, without complications
1.9
10.8
197
1221
103
Kela drug reimbursment
Diabetes, insulin-treated
1.9
10.7
215
1373
L30.8
ICD-10 Finland
Other specified dermatitis
6.5
10.7
27
43
H02AB09
ATC
hydrocortisone; systemic
7.2
10.7
25
36
N18.9
ICD-10 Finland
Chronic kidney disease, unspecified
2.7
10.2
70
276
A28
ICPC
Limited function/disability NOS
2.0
10.1
158
921
SPAT1229
SPAT
Assessment of need for aid
2.4
10.1
87
387
T90
ICPC
Diabetes non-insulin dependent
1.9
10.1
178
1083
A10BA02
ATC
metformin; oral
1.8
10.1
214
1390
S02
ICPC
Pruritus
4.5
10.0
35
82
L04AA06
ATC
mycophenolic acid; systemic
8.9
9.8
20
23
L13.8
ICD-10 Finland
Other specified bullous disorders
55.9
9.7
11
*
R06AX27
ATC
desloratadine; oral
2.0
9.7
132
728
D07BA04
ATC
hydrocortisone and antiseptics; topical
2.5
9.6
77
332
J01FF01
ATC
clindamycin; systemic
1.9
9.5
175
1082
SPAT1222
SPAT
Counselling/guidance promoting independent living
2.0
9.4
132
736
L12.2
ICD-10 Finland
Chronic bullous disease of childhood
+∞
9.4
9
*
QXB99
NOMESCO Finland
Other procedure to treat skin wound, unspecified location
5.6
9.3
26
48
S21
ICPC
Skin texture symptom/complaint
2.2
8.9
90
437
E11.7
ICD-10 Finland
Type 2 diabetes mellitus, with multiple complications
2.7
8.8
62
247
E000003
ATC
NA
3.5
8.7
40
121
K13.79
ICD-10 Finland
Change in the oral mucosa UNS
5.6
8.7
24
44
L28.1
ICD-10 Finland
Prurigo nodularis
9.2
8.6
17
19
M05BA04
ATC
alendronic acid; oral
2.2
8.5
87
423
S17
ICPC
Abrasion/scratch/blister
4.3
8.4
30
73
L10.9
ICD-10 Finland
Pemphigus, unspecified
+∞
8.3
8
*
A10AC01
ATC
insulin (human); parenteral (insulins and analog. for injection, intermediate-acting)
2.4
8.3
69
304
E000007
ATC
NA
3.0
8.2
47
166
L98.4
ICD-10 Finland
Chronic ulcer of skin, not elsewhere classified
4.3
8.2
29
70
SPAT1254
SPAT
Administration of medicine
1.9
8.2
123
702
A10BH02
ATC
vildagliptin; oral
4.6
8.1
27
61
ZY010
NOMESCO Finland
Hospital outpatient
4.0
8.1
31
81
N18.8
ICD-10 Finland
Other chronic renal failure
3.6
8.0
35
102
L20.0
ICD-10 Finland
Besnier prurigo
3.6
8.0
34
97
I10
ICD-10 Finland
Essential (primary) hypertension
1.7
8.0
421
3468
QDB10
NOMESCO Finland
Major dressing of wound of skin of lower limb
2.7
7.9
53
206
D07CC01
ATC
betamethasone and antibiotics; topical
2.2
7.9
85
425
R53
ICD-10 Finland
Malaise and fatigue
1.8
7.8
152
947
L89
ICD-10 Finland
Decubitus ulcer and pressure area
4.5
7.7
26
60
W01
ICD-10 Finland
Fall on same level from slipping, tripping and stumbling
1.7
7.7
219
1527
R06AE09
ATC
levocetirizine; oral
2.2
7.6
81
401
R03BA02
ATC
budesonide; inhalant
2.0
7.6
105
580
SPAT1262
SPAT
Handing over of treatment supplies
1.8
7.6
135
815
I70.2
ICD-10 Finland
Atherosclerosis of arteries of extremities
2.0
7.6
97
519
E86
ICD-10 Finland
Volume depletion
3.7
7.6
31
86
E000002
ATC
NA
2.3
7.6
71
331
Z2221
NOMESCO Finland
Medical doctor
1.7
7.4
174
1147
L10.0
ICD-10 Finland
Pemphigus vulgaris
81.0
7.4
8
*
L10.2
ICD-10 Finland
Pemphigus foliaceus
+∞
7.3
7
*
EKA10
NOMESCO Finland
Biopsy of cheek
5.3
7.3
21
41
XQ802
NOMESCO Finland
Epicutan test
4.4
7.3
25
59
203
Kela drug reimbursment
Chronic asthma and similar chronic obstructive pulmonary diseases
1.8
7.2
124
739
L28
ICPC
Limited function/disability (L)
1.8
7.1
131
797
D01AC20
ATC
imidazoles/triazoles in combination with corticosteroids; topical
1.8
7.1
146
921
SBB00
NOMESCO Finland
Fotograf of mouth and teeth
3.8
7.1
28
76
XQ800
NOMESCO Finland
Prick test
3.1
7.0
37
126
RS222
NOMESCO Finland
NA
6.4
7.0
17
27
C07AB07
ATC
bisoprolol; oral
1.6
6.8
339
2700
L43.1
ICD-10 Finland
Bullous lichen planus
40.5
6.8
8
*
J01AA07
ATC
tetracycline; systemic
2.1
6.8
79
407
SPAT1149
SPAT
Suture or treatment of traumatic wound
1.9
6.7
100
567
WXQ27
NOMESCO Finland
UVB narrow spectrum light therapy
7.3
6.7
15
21
L43.9
ICD-10 Finland
Lichen planus, unspecified
5.0
6.7
20
41
I83.1
ICD-10 Finland
Varicose veins of lower extremities with inflammation
3.7
6.6
27
76
K12.10
ICD-10 Finland
Stomatitis NOS
10.2
6.6
12
12
C08CA01
ATC
amlodipine; oral
1.6
6.5
258
1944
E87.1
ICD-10 Finland
Hypo-osmolality and hyponatraemia
2.6
6.4
43
170
SPAT1137
SPAT
Taking care of patient’s/customer’s hygiene
2.2
6.4
66
324
L40.0
ICD-10 Finland
Psoriasis vulgaris
3.0
6.4
34
117
D01AE15
ATC
terbinafine; topical
2.3
6.2
53
239
D07XC01
ATC
betamethasone, combinations; topical
2.0
6.1
81
440
SPAT1250
SPAT
Comprehensive monitoring of chronic illness
1.6
6.1
198
1413
J18.9
ICD-10 Finland
Pneumonia, unspecified
1.6
6.0
190
1343
A04
ICPC
Weakness/tiredness general
1.9
6.0
96
558

Mortality – FinRegistry

Association

Association between endpoint L12_PEMPHIGOID_BULL and mortality.

Females

Parameter HR [95% CI] p-value
L12_PEMPHIGOID_BULL 1.818 [1.56, 2.12] < 0.001
Birth year 0.992 [0.98, 1.0] 0.081

During the follow-up period (1.1.1998 — 31.12.2019), 1650 out of 3117 females with L12_PEMPHIGOID_BULL died.

Males

Parameter HR [95% CI] p-value
L12_PEMPHIGOID_BULL 1.918 [1.57, 2.35] < 0.001
Birth year 0.991 [0.98, 1.0] 0.051

During the follow-up period (1.1.1998 — 31.12.2019), 1263 out of 2311 males with L12_PEMPHIGOID_BULL died.

Mortality risk

Mortality risk for people of age

years, who have L12_PEMPHIGOID_BULL.

N-year risk Females Males
1 0.151% 0.439%
5 0.984% 2.339%
10 2.302% 6.252%
15 4.504% 11.913%
20 7.97% 19.969%

Relationships between endpoints

Index endpoint: L12_PEMPHIGOID_BULL – Bullous pemphigoid

GWS hits: 2

Endpoint
Case Overlap
Survival Analysis
Genetic Correlations
Genetic Signals
N (Jaccard index)
HR [CI]
Extremity
rg [CI]
Extremity
Hits
Coloc Hits
FinRegistry
FinnGen
FinRegistry
FinnGen
FinnGen
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