Endemic pemphigus foliaceus
Synonyms: Fogo selvagem
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare autoimmune bullous skin disease characterized by the formation of subcorneal blisters mediated by autoantibodies against desmoglein 1, affecting predominantly seborrheic areas such as the head, neck, and upper trunk, but without any mucosal involvement. The lesions may be worsened by exposure to UV light and typically progress over weeks or months. The disease frequently occurs in children and young adults and is endemic to rural areas of Brazil and other countries in South America, as well as North Africa.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Endemic pemphigus foliaceus | Fogo selvagem |
| fr | Pemphigus foliacé endémique | = en |
| de | Pemphigus foliaceus, endemischer Typ | Brasilianischer Pemphigus · Fogo Selvagem |
| es | Pénfigo foliáceo endémico | = en |
| it | Pemfigo foliaceo endemico | Fuoco selvaggio |
| nl | Endemische pemphigus foliaceus | = en |
| pt | Pênfigo foliáceo endémico | = en |
| pl | Endemiczna pęcherzyca liściasta | = en |
| cs | Endemický pemphigus foliaceus | = en |
| uk | Ендемічна листоподібна пухирчатка | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L10.3 | E | specific | validated |
| ICD-11 | EB40.1 | NTBT | index | validated |
| MONDO | 0043257 | E | validated | |
| MONDO | 43257 | E | validated | |
| MeSH | C535551 | E | validated | |
| UMLS | C0263314 | 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.4 / 100 000 | Tunisia | Value and class | Validated | 34909708[PMID] |
| Point prevalence | Unknown | — | Europe | Class only | Validated | 34909708[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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/636955
- This record as JSON: /data/d/636955.json