Dermatitis herpetiformis
General Information (adopted from Orphanet):
Synonyms, Signs: |
DH Durhing-Brocq disease |
Number of Symptoms | 17 |
OrphanetNr: | 1656 |
OMIM Id: |
601230
|
ICD-10: |
L13.0 |
UMLs: |
C0011608 |
MeSH: |
D003874 |
MedDRA: |
10012468 |
Snomed: |
111196000 |
Prevalence, inheritance and age of onset:
Prevalence: | 27 of 100 000 [Orphanet] |
Inheritance: |
Not applicable [Orphanet] |
Age of onset: |
All ages [Orphanet] |
Disease classification (adopted from Orphanet):
Parent Diseases: |
Autoimmune bullous skin disease
-Rare skin disease |
Symptom Information:
|
(HPO:0002653) | Bone pain | Occasional [Orphanet] | 75 / 7739 | |||
|
(HPO:0002659) | Increased susceptibility to fractures | Very frequent [Orphanet] | 110 / 7739 | |||
|
(HPO:0002024) | Malabsorption | Very frequent [Orphanet] | 142 / 7739 | |||
|
(HPO:0001025) | Urticaria | Very frequent [Orphanet] | 73 / 7739 | |||
|
(HPO:0000964) | Eczema | Frequent [Orphanet] | 81 / 7739 | |||
|
(HPO:0200037) | Skin vesicle | Very frequent [Orphanet] | 102 / 7739 | |||
|
(HPO:0000989) | Pruritus | Very frequent [Orphanet] | 24068131 | IBIS | 111 / 7739 | |
|
(HPO:0010783) | Erythema | Very frequent [Orphanet] | 138 / 7739 | |||
|
(HPO:0100725) | Lichenification | Occasional [Orphanet] | 14 / 7739 | |||
|
(HPO:0001935) | Microcytic anemia | Very frequent [Orphanet] | 32 / 7739 | |||
|
(HPO:0000969) | Edema | Occasional [Orphanet] | 117 / 7739 | |||
|
(HPO:0002960) | Autoimmunity | Very frequent [Orphanet] | 78 / 7739 | |||
|
(OMIM) | Associated with HLA DQA1*0501 (146880) and B1*0201 (142857) | 1 / 7739 | ||||
|
(HPO:0000006) | Autosomal dominant inheritance | 2518 / 7739 | ||||
|
(HPO:0030350) | Erythematous papule | Very frequent [Orphanet] | 123 / 7739 | |||
|
(MedDRA:10012468) | Dermatitis herpetiformis | 2 / 7739 | ||||
|
(MedDRA:10025421) | Macule | Very frequent [Orphanet] | 55 / 7739 |
Associated genes:
ClinVar (via SNiPA)
Gene symbol | Variation | Clinical significance | Reference |
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