Pemphigus vegetans
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pemphigus vegetans | |
| fr | Pemphigus végétant | |
| de | Pemphigus vegetans | |
| es | Pénfigo vegetativo | |
| it | Pemfigo vegetante | |
| nl | Pemphigus vegetans | |
| pt | Pênfigo vegetante | |
| pl | Pęcherzyca bujająca | |
| cs | Pemphigus vegetans | |
| uk | Пемфігус вегетуючий | |
| zh | 增殖型天疱疮 | |
| tr | Pemfigus vejetans |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L10.1 | E | specific | validated |
| ICD-11 | EB40.0Y | NTBT | index | validated |
| MONDO | 0019322 | E | validated | |
| UMLS | C0263316 | E | validated | |
| MedDRA | 10057053 | 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:79669Autoimmune bullous skin disease
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 / 1 000 000 | 0.391 / 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
25 annotations · validated 2026-05-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:4000013 | Anti-desmoglein-1 antibody positivity | |
| HP:4000014 | Anti-desmoglein-3 antibody positivity | |
| Frequent (79-30%) | ||
| HP:0000159 | Abnormal lip morphology | |
| HP:0030809 | Abnormal tongue morphology | |
| HP:6000448 | Anti-desmocollin antibody positivity | |
| HP:6000956 | Exuberant granulation tissue | |
| HP:0000962 | Hyperkeratosis | |
| HP:0033802 | Intra-epidermal blistering | |
| HP:0032007 | Maceration | |
| HP:0200097 | Oral mucosal blisters | |
| HP:0012531 | Pain | |
| HP:0033700 | Papulovesicular eruption | |
| HP:0200039 | Pustule | |
| HP:0200041 | Skin erosion | |
| HP:0200035 | Skin plaque | |
| HP:0200042 | Skin ulcer | |
| HP:0010280 | Stomatitis | |
| HP:0012500 | Verrucous papule | |
| HP:0033697 | Vesicular eruption | |
| Occasional (29-5%) | ||
| HP:0001597 | Abnormal nail morphology | |
| HP:0025757 | Abnormal nail surface | |
| HP:0001808 | Fragile nails | |
| HP:0004395 | Malnutrition | |
| HP:0025088 | Onychomadesis | |
| HP:0001818 | Paronychia | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 31365654[PMID]_31424813[PMID]_260510641[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/79479
- This record as JSON: /data/d/79479.json