Pemphigus erythematosus
Synonyms: Seborrheic pemphigus · Senear-Usher syndrome
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare superficial pemphigus disease characterized clinically by well-demarcated, localized, erythematous, scaly, hyperkeratotic, crusted plaques, with frequent butterfly distribution over the malar area of the face (but also commonly involving trunk and scalp, and less frequently the extremities, with a photoexposed distribution). Histologically, granular deposits along the dermal-epidermal junction, in addition to intercellular deposition in the upper epidermis, are observed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pemphigus erythematosus | Seborrheic pemphigus · Senear-Usher syndrome |
| fr | Pemphigus érythémateux | Pemphigus séborrhéique · Syndrome de Senear-Usher |
| de | Pemphigus erythematosus | Pemphigus seborrhoicus · Senear-Usher-Syndrom |
| es | Pénfigo eritematoso | Pénfigo seborreico · Síndrome de Senear-Usher |
| it | Pemfigo eritematoso | Pemfigo seborroico · Sindrome di Senear-Usher |
| nl | Pemphigus erythematosus | Erythemateuze pemfigus · Pemphigus seborrhoicus · Seborroïsche pemfigus · Syndroom van Senear-Usher |
| pt | Pênfigo eritematoso | Pênfigo seborréico · Sííndrome Senear-Usher |
| pl | Pęcherzyca rumieniowata | Seborrheic pemphigus · Zespół Seneara i Ushera |
| cs | Pemphigus erythematosus | Pemphigus seborrhoicus · Senearův-Usherův syndrom · Senear-Usherův syndrom |
| uk | Червоний вовчак | Себорейна пухирниця · Синдром Сенера-Ушера |
| zh | 红斑型天疱疮 | Senear-Usher综合征 |
| tr | Pemfigus eritematozus | Seboreik pemfigus · Senear-Usher sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L10.4 | NTBT | attributed | validated |
| ICD-11 | EB40.1 | NTBT | index | validated |
| MONDO | 0019323 | E | validated | |
| UMLS | C0263312 | E | validated | |
| MedDRA | 10058917 | 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:46485Superficial pemphigus
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 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.
HPO phenotypes
14 annotations · validated 2021-11-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0100792 | Acantholysis | |
| HP:0002960 | Autoimmunity | |
| HP:0025474 | Erythematous plaque | |
| HP:0011355 | Localized skin lesion | |
| HP:0025300 | Malar rash | |
| Frequent (79-30%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0031540 | Linear IgG deposits along the epidermal basement membrane zone | |
| Occasional (29-5%) | ||
| HP:0030208 | Acetylcholine receptor antibody positivity | |
| HP:0007510 | Focal dermal aplasia/hypoplasia | |
| HP:0001053 | Hypopigmented skin patches | |
| HP:0000155 | Oral ulcer | |
| HP:0200041 | Skin erosion | |
| HP:0002725 | Systemic lupus erythematosus | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 17377385[PMID]_31396635[PMID]_26814382[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/79480
- This record as JSON: /data/d/79480.json