ORPHA:46488
Linear IgA dermatosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare, acquired autoimmune bullous skin disease characterized by annular, grouped blisters on the skin and, frequently, mucous membranes with linear deposition of immunoglobulin A along the basement membrane zone (BMZ).
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Linear IgA dermatosis | |
| fr | Dermatose à IgA linéaire | |
| de | IgA-Dermatose, lineare | |
| es | Dermatosis IgA linear | |
| it | Dermatosi da IgA lineari | |
| nl | Lineaire IgA-dermatose | |
| pt | Dermatose por acumulação linear de IgA | |
| pl | Linijna dermatoza IgA | |
| cs | Lineární IgA bulózní dermatóza | |
| uk | Лінійний IgA дерматоз | |
| zh | 线性IgA皮肤病 | |
| tr | Lineer IgA dermatoz |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L13.8 | NTBT | attributed | validated |
| ICD-11 | EB42 | E | specific | validated |
| MONDO | 0018748 | E | validated | |
| MeSH | D062027 | E | validated | |
| UMLS | C0406650 | E | validated | |
| MedDRA | 10024515 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:79669Autoimmune bullous skin disease
rare skin diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
6 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.1 / 100 000 | Germany | Value and class | Validated | 19170813[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.069 / 100 000 | Kuwait | Value and class | Validated | 15569006[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.026 / 100 000 | Singapore | Value and class | Validated | 12207586[PMID]_23237497[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.3 / 100 000 | Germany | Value and class | Validated | 19170813[PMID]_30252369[PMID]_ORPHANET |
| Point prevalence | 1-9 / 1 000 000 | 0.207 / 100 000 | Kuwait | Value and class | Validated | 15569006[PMID]_30252369[PMID]_ORPHANET |
| Point prevalence | <1 / 1 000 000 | 0.078 / 100 000 | Singapore | Value and class | Validated | 12207586[PMID]_23237497[PMID]_30252369[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
6
HPO phenotypes
11 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0002960 | Autoimmunity | |
| HP:0200034 | Papule | |
| Frequent (79-30%) | ||
| HP:4000028 | Anti-LABD97 antibody positivity | |
| HP:0000155 | Oral ulcer | |
| HP:4000027 | anti-LAD-1 antibody positivity | |
| Occasional (29-5%) | ||
| HP:0009725 | Bladder neoplasm | |
| HP:0000421 | Epistaxis | |
| HP:0002037 | Inflammation of the large intestine | |
| HP:0000989 | Pruritus | |
| HP:0009726 | Renal neoplasm | |
Run the differential on the 3 obligate / very frequent terms
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/46488
- This record as JSON: /data/d/46488.json