Dermatitis herpetiformis

L12_DERMATHERP

dermatitis herpetiformis: Dermatitis herpetiformis (DH) is a chronic autoimmune subepidermal bullous disease characterized by grouped pruritic lesions such as papules, urticarial plaques, erythema, and herpetiform vesiculae, with a predominantly symmetrical distribution on extensor surfaces of the elbows (90%), knees (30%), shoulders, buttocks, sacral region, and face of children and adults. Erosions, excoriations and hyperpigmentation usually follow. DH may also appear as a consequence of gluten intolerance.

Suggest a new description

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 L13.0
  • Hospital discharge: ICD-9 6940A|6942
  • Hospital discharge: ICD-8 693
  • Cause of death: ICD-10 L13.0
  • Cause of death: ICD-9 6940A|6942
  • Cause of death: ICD-8 693

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

661

4. Check minimum number of events

None

661

5. Include endpoints

None

661

6. Filter based on genotype QC (FinnGen only)

661

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
L13
Name in latin
Dermatitis herpetiformis

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 4875 2511 2338
Only index persons 4281 2269 2012
Unadjusted period prevalence (%)
Whole population 0.07 0.07 0.07
Only index persons 0.08 0.08 0.08
Median age at first event (years)
Whole population 45.74 45.97 45.38
Only index persons 44.15 44.66 43.57

-FinnGen-

Key figures

All Female Male
Number of individuals 661 378 283
Unadjusted period prevalence (%) 0.13 0.13 0.12
Median age at first event (years) 45.33 41.57 50.36

-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
601
Matched controls
6010
Code
Vocabulary
Description
Odds Ratio
-log10(p)
N matched cases
N matched controls
L13.0
ICD-10 Finland
Dermatitis herpetiformis
16384.0
323.0
492
*
K90.0
ICD-10 Finland
Coeliac disease
86.6
264.3
288
63
J04BA02
ATC
dapsone; oral
368.5
188.6
181
7
69399
ICD-8 Finland
Dermatitis herpetiformis
+∞
123.2
114
*
TQX10
NOMESCO Finland
Biopsy of skin and subcutaneous tissue
8.1
75.7
191
325
UJD10
NOMESCO Finland
Esophagoscopy, gastroscopy and duodenoscopy
3.7
46.8
284
1165
D07AC01
ATC
betamethasone; topical
3.4
42.7
306
1404
L30.9
ICD-10 Finland
Dermatitis, unspecified
3.9
38.2
186
613
D07BB04
ATC
hydrocortisone butyrate and antiseptics; topical
4.1
33.3
146
432
D07AC13
ATC
mometasone; topical
3.0
31.6
245
1119
E000001
ATC
NA
3.3
30.1
189
741
D07AB02
ATC
hydrocortisone butyrate; topical
2.9
29.1
230
1050
6940A
ICD-9 Finland
Bullous dermatoses, Dermatitis herpetiformis
+∞
28.3
27
*
D07AB08
ATC
desonide; topical
4.1
25.0
107
304
E0000UV
ATC
NA
2.4
23.1
285
1625
QCA99
NOMESCO Finland
Other local destruction of skin of upper limb
59.6
20.0
23
*
L20.0
ICD-10 Finland
Besnier prurigo
4.6
18.2
65
154
D07BA04
ATC
hydrocortisone and antiseptics; topical
3.3
17.7
95
321
D07CC01
ATC
betamethasone and antibiotics; topical
2.9
16.4
112
445
L12.0
ICD-10 Finland
Bullous pemphigoid
+∞
15.7
15
*
E000002
ATC
NA
2.9
15.5
100
382
D07AD01
ATC
clobetasol; topical
2.6
15.2
125
553
A12AX
ATC
Calcium, combinations with vitamin D and/or other drugs
2.1
14.7
218
1281
E000007
ATC
NA
4.1
13.8
54
140
K90.9
ICD-10 Finland
Intestinal malabsorption, unspecified
29.1
13.0
17
6
H02AB06
ATC
prednisolone; systemic
1.9
12.6
293
2012
D07XB05
ATC
dexamethasone, combinations; topical
5.7
12.4
35
64
L13.9
ICD-10 Finland
Bullous disorder, unspecified
18.5
12.1
18
10
R06AE07
ATC
cetirizine; oral
1.8
11.6
272
1855
R06AE09
ATC
levocetirizine; oral
2.4
11.5
108
506
D07AA02
ATC
hydrocortisone; topical
2.4
10.8
99
458
RD210
NOMESCO Finland
NA
6.9
10.8
26
39
R06AX13
ATC
loratadine; oral
2.3
10.7
101
476
D07BC01
ATC
betamethasone and antiseptics; topical
2.4
10.3
92
421
Z71.3
ICD-10 Finland
Dietary counselling and surveillance
3.4
9.9
48
151
317
Kela drug reimbursment
Topical pimecrolimus and tacrolimus
8.9
9.8
20
23
Z01.5
ICD-10 Finland
Diagnostic skin and sensitization tests
2.5
9.5
73
309
69300
ICD-8 Finland
NA
+∞
9.4
9
*
N05BB01
ATC
hydroxyzine; systemic
3.0
8.6
49
173
S02
ICPC
Pruritus
3.9
8.2
32
85
SPAT1263
SPAT
Sampling
1.7
8.1
254
1830
203
Kela drug reimbursment
Chronic asthma and similar chronic obstructive pulmonary diseases
1.9
8.0
118
671
J05AB01
ATC
aciclovir; systemic
1.9
7.8
130
766
TQW80
NOMESCO Finland
Local dermatologic skincare
4.2
7.8
28
69
J01AA07
ATC
tetracycline; systemic
2.1
7.6
95
503
E000006
ATC
NA
2.6
7.4
52
211
L13.1
ICD-10 Finland
Subcorneal pustular dermatitis
+∞
7.3
7
*
D11AX14
ATC
[U] tacrolimus; topical
20.3
7.1
10
5
D07CA01
ATC
hydrocortisone and antibiotics; topical
2.0
7.1
88
466
D01AC20
ATC
imidazoles/triazoles in combination with corticosteroids; topical
1.8
7.1
137
852
D07AC14
ATC
methylprednisolone aceponate; topical
3.3
7.1
34
108
D07XC01
ATC
betamethasone, combinations; topical
2.1
7.0
81
417
M35.0
ICD-10 Finland
Sicca syndrome [Sjögren]
5.1
6.8
20
40
D11AH01
ATC
tacrolimus; topical
9.5
6.8
13
14
SPAT1249
SPAT
Treatment-related counselling outside office
1.6
6.8
232
1692
S01XA20
ATC
artificial tears and other indifferent preparations; ophthalmic
1.6
6.8
233
1698
J01DA01
ATC
[U] cefalexin; systemic
1.6
6.7
361
2940
D09AB01
ATC
zinc bandage without supplements; topical
4.3
6.7
23
55
WXQ27
NOMESCO Finland
UVB narrow spectrum light therapy
6.0
6.6
17
29
L30.3
ICD-10 Finland
Infective dermatitis
3.2
6.6
32
103
L29.9
ICD-10 Finland
Pruritus, unspecified
4.2
6.6
23
56
L08.8
ICD-10 Finland
Other specified local infections of skin and subcutaneous tissue
4.2
6.6
23
56
J45.0
ICD-10 Finland
Predominantly allergic asthma
2.4
6.5
52
226
L28.1
ICD-10 Finland
Prurigo nodularis
7.5
6.4
14
19
D02AX
ATC
Other emollients and protectives
3.3
6.1
28
87

Mortality – FinRegistry

Association

Association between endpoint L12_DERMATHERP and mortality.

Females

Parameter HR [95% CI] p-value
L12_DERMATHERP 0.983 [0.85, 1.14] 0.828
Birth year 0.994 [0.99, 1.0] 0.152

During the follow-up period (1.1.1998 — 31.12.2019), 253 out of 1738 females with L12_DERMATHERP died.

Males

Parameter HR [95% CI] p-value
L12_DERMATHERP 0.935 [0.82, 1.07] 0.33
Birth year 0.988 [0.98, 1.0] 0.011

During the follow-up period (1.1.1998 — 31.12.2019), 281 out of 1496 males with L12_DERMATHERP died.

Mortality risk

Mortality risk for people of age

years, who have L12_DERMATHERP.

N-year risk Females Males
1 0.093% 0.172%
5 0.593% 1.034%
10 1.418% 2.787%
15 2.79% 5.294%
20 4.949% 8.632%

Relationships between endpoints

Index endpoint: L12_DERMATHERP – Dermatitis herpetiformis

GWS hits: 3

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
loading spinner Loading data