Dermatitis herpetiformis
Synonyms: Duhring-Brocq disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
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. It may also appear as a consequence of gluten intolerance.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Dermatitis herpetiformis | Duhring-Brocq disease |
| fr | Dermatite herpétiforme | Maladie de Duhring-Brocq |
| de | Dermatitis herpetiformis | Dermatitis herpetiformis Duhring · Duhring-Brocq-Krankheit |
| es | Dermatitis herpetiforme | Enfermedad de Durhing-Brocq |
| it | Dermatite erpetiforme | Malattia di Duhring-Brocq |
| nl | Dermatitis herpetiformis | Ziekte van Duhring-Brocq |
| pt | Dermatite herpetiforme | Doença de Duhring-Brocq |
| pl | Opryszczkowate zapalenie skóry | Choroba Durhinga i Brocqa |
| cs | Duhringova dermatitida | Duhringova choroba · Duhringova herpetiformní dermatitida · Dermatitis herpetiformis Duhring · Duhringova dermatitida |
| uk | Герпетиформний дерматит | Хвороба Дюринга-Брока |
| zh | 疱疹样皮炎 | Duhring-Brocq病 |
| tr | Dermatit herpetiformis | Duhring-Brocq hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 601230 | BTNT | validated | |
| ICD-10 | L13.0 | E | specific | validated |
| ICD-11 | EB44 | E | specific | validated |
| MONDO | 0015614 | E | validated | |
| MeSH | D003874 | E | validated | |
| UMLS | C0011608 | E | validated | |
| GARD | 1917 | E | validated | |
| MedDRA | 10012468 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:79669Autoimmune bullous skin disease
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
11 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 27 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Annual incidence | 1-9 / 1 000 000 | 0.9 / 100 000 | Italy | Value and class | Validated | [EXPERT] |
| Annual incidence | 1-9 / 100 000 | 1.5 / 100 000 | Ireland | Value and class | Validated | 5023901[PMID]_6641006[PMID]_[EXPERT]_ORPHANET |
| Point prevalence | 1-5 / 10 000 | 17.6 / 100 000 | Ireland | Value and class | Validated | 5023901[PMID]_6641006[PMID]_[EXPERT]_ORPHANET |
| Annual incidence | 1-9 / 100 000 | 1.1 / 100 000 | Sweden | Value and class | Validated | 6208718[PMID] |
| Point prevalence | 1-5 / 10 000 | 21 / 100 000 | Sweden | Value and class | Not yet validated | 6208718[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.98 / 100 000 | United States | Value and class | Validated | 1456754[PMID] |
| Point prevalence | 1-5 / 10 000 | 11.2 / 100 000 | United States | Value and class | Validated | 1456754[PMID] |
| Annual incidence | 1-9 / 100 000 | 3.5 / 100 000 | Finland | Value and class | Validated | 21517799[PMID] |
| Point prevalence | 6-9 / 10 000 | 75.3 / 100 000 | Finland | Value and class | Validated | 21517799[PMID] |
| Point prevalence | 1-5 / 10 000 | 11.5 / 100 000 | United Kingdom | Value and class | Validated | 6693042[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
26 annotations · validated 2023-09-15| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0002960 | Autoimmunity | |
| HP:0010783 | Erythema | |
| HP:0012538 | Gluten intolerance | |
| HP:0012733 | Macule | |
| HP:0002024 | Malabsorption | |
| HP:0001935 | Microcytic anemia | |
| HP:0000989 | Pruritus | |
| HP:0002757 | Recurrent fractures | |
| HP:0200037 | Skin vesicle | |
| HP:0001025 | Urticaria | |
| Frequent (79-30%) | ||
| HP:0033637 | Anti-endomysial antibody positivity | |
| HP:0033565 | Anti-epidermal transglutaminase antibody positivity | |
| HP:4000030 | Anti-reticulin antibody positivity | |
| HP:4000026 | Anti-transglutaminase 6 antibody | |
| HP:4000031 | Anti-type VII collagen antibody | |
| HP:4000029 | Antigliadin antibody positivity | |
| HP:0000964 | Eczematoid dermatitis | |
| Occasional (29-5%) | ||
| HP:0000820 | Abnormality of the thyroid gland | |
| HP:0002653 | Bone pain | |
| HP:0000684 | Delayed eruption of teeth | |
| HP:0009722 | Dental enamel pits | |
| HP:0000969 | Edema | |
| HP:0100725 | Lichenification | |
| Very rare (<4-1%) | ||
| HP:0000707 | Abnormality of the nervous system | |
| HP:0031446 | Erosion of oral mucosa | |
Run the differential on the 11 obligate / very frequent terms
Annotation source: 29630215[PMID]_34441049[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1656
- This record as JSON: /data/d/1656.json