Dermatitis herpetiformis

Description

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.

Data source
FinnGen phenocode L12_DERMATHERP
Hospital Discharge registry ICD-10: L130, ICD-9: 6940A, ICD-8: 693
Cause of Death registry ICD-10: L130, ICD-9: 6940A, ICD-8: 693
Level in the ICD-hierarchy 4
First defined in version DF2
Ontology
DOID 8505
GWAS catalog 1000684
MESH D003874
SNOMED CT 111196000

Key figures

Sex All Female Male
Number of individuals 173 103 70
Unadjusted prevalence (%) 0.13 0.14 0.12
Mean age at first event (years) 44.86 42.45 48.41
Median number of events / individual 2 1 3

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 41.04 35.92 48.57
Case fatality at 5-years (%) 1.73 0.97 2.86

Associations