Pemphigus foliaceus
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
A rare superficial pemphigus disease characterized by multiple, pruritic, scaly, crusted cutaneous erosions, with flaky circumscribed patches, localized mostly on the face, scalp, trunk and extremities, often presenting an erythematous base. Mucosal involvement is rarely observed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pemphigus foliaceus | |
| fr | Pemphigus foliacé | |
| de | Pemphigus foliaceus | |
| es | Pénfigo foliáceo | |
| it | Pemfigo foliaceo | |
| nl | Pemphigus foliaceus | |
| pt | Pênfigo foliáceo | |
| pl | Pęcherzyca liściasta | |
| cs | Pemphigus foliaceus | |
| uk | Пемфігоїд рубцевий | |
| zh | 落叶型天疱疮 | |
| tr | Pemfigus foliaceus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L10.2 | NTBT | attributed | validated |
| ICD-11 | EB40.1 | E | specific | validated |
| MONDO | 0019324 | E | validated | |
| UMLS | C0263313 | E | validated | |
| GARD | 7354 | E | validated | |
| MedDRA | 10057069 | 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 | 1-9 / 100 000 | 1.001 / 100 000 | Germany | Value and class | Validated | 27456755[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
20 annotations · validated 2021-11-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0100792 | Acantholysis | |
| HP:0002960 | Autoimmunity | |
| Frequent (79-30%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0001965 | Abnormality of the scalp | |
| HP:0007473 | Crusting erythematous dermatitis | |
| HP:0010783 | Erythema | |
| HP:0025474 | Erythematous plaque | |
| HP:0000989 | Pruritus | |
| HP:0040189 | Scaling skin | |
| HP:0200041 | Skin erosion | |
| Occasional (29-5%) | ||
| HP:0011830 | Abnormal oral mucosa morphology | |
| HP:0001019 | Erythroderma | |
| HP:0000155 | Oral ulcer | |
| HP:0200037 | Skin vesicle | |
| Very rare (<4-1%) | ||
| HP:0025528 | Annular cutaneous lesion | |
| HP:0004377 | Hematological neoplasm | |
| HP:0008069 | Neoplasm of the skin | |
| HP:0003765 | Psoriasiform dermatitis | |
| HP:0200039 | Pustule | |
| HP:0025527 | Serpiginous cutaneous lesion | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 31378971[PMID]_29479654[PMID]_30762246[PMID]_29267466[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/79481
- This record as JSON: /data/d/79481.json