Generalized pustular psoriasis

L12_PSORI_PUSTGEN

impetigo herpetiformis: An impetigo that is characterized as a form of severe pustular psoriasis occurring in pregnancy.

Suggest a new description

Endpoint definition

Endpoint definition steps

FinnGen

Phenotype data

473681

1. Apply sex-specific rule

None

473681

2. Check conditions

None

473681

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

Registry filters:

  • Hospital discharge: ICD-10 L40.1
  • Hospital discharge: ICD-9 6961B
  • Hospital discharge: ICD-8 69610
  • Cause of death: ICD-10 L40.1
  • Cause of death: ICD-9 6961B
  • Cause of death: ICD-8 69610

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

113

4. Check minimum number of events

None

113

5. Include endpoints

None

113

6. Filter based on genotype QC (FinnGen only)

108

Control definitions (FinnGen only)

Control exclude
L12_PAPULOSQUAMOUS

Extra metadata

Level in the ICD hierarchy
4
First used in FinnGen datafreeze
DF2
Parent code in ICD-10
L40
Name in latin
Psoriasis pustulosa generalisata

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 983 481 487
Only index persons 740 384 356
Unadjusted period prevalence (%)
Whole population 0.01 0.01 0.01
Only index persons 0.01 0.01 0.01
Median age at first event (years)
Whole population 54.30 56.74 51.88
Only index persons 52.55 54.23 50.72

-FinnGen-

Key figures

All Female Male
Number of individuals 108 56 52
Unadjusted period prevalence (%) 0.02 0.02 0.03
Median age at first event (years) 51.85 49.45 54.42

-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

No data

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
113
Matched controls
1128
Code
Vocabulary
Description
Odds Ratio
-log10(p)
N matched cases
N matched controls
L40.1
ICD-10 Finland
Generalized pustular psoriasis
+∞
122.0
94
*
L40.0
ICD-10 Finland
Psoriasis vulgaris
288.9
68.8
64
5
D05AX02
ATC
calcipotriol; topical
157.6
52.3
52
6
D05BB02
ATC
acitretin; oral
158.2
47.2
47
5
D05AX52
ATC
calcipotriol, combinations; topical
57.3
45.6
53
17
D07XC01
ATC
betamethasone, combinations; topical
25.9
42.1
63
52
L40.5
ICD-10 Finland
Arthropathic psoriasis
+∞
38.8
35
*
L04AX03
ATC
methotrexate; systemic (immunosuppressants)
21.9
33.8
52
42
D07AC01
ATC
betamethasone; topical
13.2
32.9
89
247
M07.3*L40.5
ICD-10 Finland
Other psoriatic arthropathies
+∞
31.8
29
*
E0000UV
ATC
NA
12.1
30.0
92
299
D07AD01
ATC
clobetasol; topical
13.2
29.4
59
86
D05AX03
ATC
calcitriol; topical
350.6
28.0
27
*
E000001
ATC
NA
10.0
26.3
65
134
L40.9
ICD-10 Finland
Psoriasis, unspecified
317.3
25.8
25
*
319
Kela drug reimbursment
Adalimumab, brodalumab, certolizumab pegol, etanercept, guselkumab, infliximab, ixekizumab, risankizumab, sekukinumab and ustekinumab (skin psoriasis)
+∞
23.8
22
*
TQX10
NOMESCO Finland
Biopsy of skin and subcutaneous tissue
13.1
20.6
38
42
134
Kela drug reimbursment
General erythroderma
+∞
20.4
19
*
E000003
ATC
NA
18.9
20.1
31
22
L40.3
ICD-10 Finland
Pustulosis palmaris et plantaris
113.1
18.2
19
*
202
Kela drug reimbursment
Connective tissue diseases, rheumatoid arthritis and comparable disease
8.7
17.0
40
67
L40.8
ICD-10 Finland
Other psoriasis
198.0
17.0
17
*
L01BA01
ATC
methotrexate; systemic (folic acid analog.)
23.0
16.9
24
13
L04AB04
ATC
adalimumab; parenteral
36.3
16.8
21
7
281
Kela drug reimbursment
Abatacept, adalimumab, anakinra, certolizumab pegol, etanercept, golimumab, infliximab, ixekizumab, sarilumab, secukinumab and tocilizumab
29.9
15.3
20
8
313
Kela drug reimbursment
Abatacept, adalimumab, anakinra, certolizumab pegol, etanercept, golimumab, infliximab, ixekizumab, sarilumab, secukinumab, tocilizumab and ustekinumab (rheumatoid diseases)
26.6
14.8
20
9
D07AB02
ATC
hydrocortisone butyrate; topical
5.3
14.7
59
194
D07AC13
ATC
mometasone; topical
4.9
14.1
62
223
TQW80
NOMESCO Finland
Local dermatologic skincare
20.4
13.0
19
11
D07AB08
ATC
desonide; topical
7.6
12.9
32
56
L04AD01
ATC
ciclosporin; systemic
30.7
12.4
16
6
E000002
ATC
NA
6.2
10.9
31
65
L04AC10
ATC
secukinumab; parenteral
120.8
10.6
11
*
6961A
ICD-9 Finland
Psoriasis and similar disorders, Other psoriasis[PSORIASIS VULGARIS]
+∞
10.6
10
*
M07.3
ICD-10 Finland
Other psoriatic arthropathies
+∞
10.6
10
*
L04AB01
ATC
etanercept; parenteral
44.2
10.2
12
*
A07EC01
ATC
sulfasalazine; oral, rectal
7.5
10.1
24
39
H02AB06
ATC
prednisolone; systemic
3.6
9.7
75
397
6961B
ICD-9 Finland
Psoriasis and similar disorders, Other psoriasis[PSORIASIS PUSTULARIS]
+∞
9.5
9
*
69610
ICD-8 Finland
Psoriasis and similar disorders, Psoriasis pustulosa
+∞
9.5
9
*
M07.0*L40.5
ICD-10 Finland
Distal interphalangeal psoriatic arthropathy
+∞
9.5
9
*
NK4XE
NOMESCO Finland
Joint ultrasound examination not elsewhere classified
8.1
9.4
21
31
L04AA01
ATC
[U] ciclosporin; systemic
26.6
9.1
12
5
L04AC05
ATC
ustekinumab; parenteral
54.3
8.8
10
*
D02AX
ATC
Other emollients and protectives
18.1
8.8
13
8
WXQ27
NOMESCO Finland
UVB narrow spectrum light therapy
30.1
8.7
11
*
TNX10
NOMESCO Finland
Arthrocentesis, unspecified region, unspecified region
7.1
8.0
19
31
L30.9
ICD-10 Finland
Dermatitis, unspecified
4.1
7.8
33
104
L27.0
ICD-10 Finland
Generalized skin eruption due to drugs and medicaments
27.2
7.7
10
*
D07AA02
ATC
hydrocortisone; topical
4.7
7.7
27
71
69619
ICD-8 Finland
Psoriasis and similar disorders, Psoriasis alia sive NUD (vulgaris)
+∞
7.4
7
*
6960A
ICD-9 Finland
Psoriasis and similar disorders, Psoriatic arthropathy
+∞
7.4
7
*
ND2AA
NOMESCO Finland
Hand and fingers X-ray examination
5.5
6.6
19
40
S91
ICPC
Psoriasis
73.8
6.5
7
*
D05AA
ATC
Tars
73.8
6.5
7
*
L40
ICD-10 Finland
Psoriasis
28.4
6.3
8
*
L04AC13
ATC
ixekizumab; parenteral
+∞
6.3
6
*
E000007
ATC
NA
6.7
6.3
15
25

Mortality – FinRegistry

Association

Association between endpoint L12_PSORI_PUSTGEN and mortality.

Females

Parameter HR [95% CI] p-value
L12_PSORI_PUSTGEN 2.109 [1.55, 2.87] < 0.001
Birth year 0.988 [0.98, 1.0] 0.011

During the follow-up period (1.1.1998 — 31.12.2019), 84 out of 289 females with L12_PSORI_PUSTGEN died.

Males

Parameter HR [95% CI] p-value
L12_PSORI_PUSTGEN 2.046 [1.29, 3.24] 0.002
Birth year 0.988 [0.98, 1.0] 0.01

During the follow-up period (1.1.1998 — 31.12.2019), 89 out of 268 males with L12_PSORI_PUSTGEN died.

Mortality risk

Mortality risk for people of age

years, who have L12_PSORI_PUSTGEN.

N-year risk Females Males
1 0.185% 0.352%
5 0.897% 2.262%
10 2.342% 5.795%
15 4.631% 11.36%
20 8.298% 18.627%

Relationships between endpoints

Index endpoint: L12_PSORI_PUSTGEN – Generalized pustular psoriasis

GWS hits: -

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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