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.

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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 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.

601

4. Check minimum number of events

None

601

5. Include endpoints

None

601

6. Filter based on genotype QC (FinnGen only)

585

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 585 333 252
Unadjusted period prevalence (%) 0.13 0.13 0.13
Median age at first event (years) 45.47 41.92 50.16

-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

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: 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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