Hydroa vacciniforme
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare photodermatosis characterized by the development of pruritic or painful vesicles in a photodistributed pattern in response to sunlight exposure. The lesions heal with permanent varioliform scarring. Ocular involvement, deformities of ears and nose, or contractures of the fingers may occasionally be observed. Systemic signs and symptoms are absent. The condition typically occurs in childhood and regresses spontaneously in adolescence or young adulthood.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hydroa vacciniforme | |
| fr | Hydroa vacciniforme | |
| de | Hydroa vacciniforme | |
| es | Hidroa vacciniforme | |
| it | Idroa vacciniforme | |
| nl | Hydroa vacciniforme | |
| pt | Hidroa vaciniforme | |
| pl | Opryszczka ospówkowata | |
| cs | Hydroa vacciniforme | |
| uk | Hydroa vacciniforme | |
| zh | 种痘样水疱病 | |
| tr | Hidroa vaksiniforme |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L56.4 | NTBT | index | validated |
| ICD-11 | EJ30.Y | NTBT | index | validated |
| MONDO | 0018024 | E | validated | |
| MeSH | D006837 | E | validated | |
| UMLS | C0020241 | E | validated | |
| GARD | 9654 | E | validated | |
| MedDRA | 10083442 | 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:79390Rare photodermatosis
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 |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 1 000 000 | 0.34 / 100 000 | United Kingdom | Value and class | Validated | 10642674[PMID] |
| Point prevalence | 1-9 / 1 000 000 | — | Europe | Class only | Not yet 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
18 annotations · validated 2023-04-13| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0000992 | Cutaneous photosensitivity | |
| HP:0032381 | Hydroa vacciniforme | |
| HP:0100699 | Scarring | |
| Very frequent (99-80%) | ||
| HP:0025300 | Malar rash | |
| HP:0033700 | Papulovesicular eruption | |
| Occasional (29-5%) | ||
| HP:0000964 | Eczematoid dermatitis | |
| HP:0009926 | Epiphora | |
| HP:0010783 | Erythema | |
| HP:0002315 | Headache | |
| HP:0000491 | Keratitis | |
| HP:0031541 | Linear C3 deposits along the epidermal basement membrane zone | |
| HP:0033834 | Malaise | |
| HP:0000613 | Photophobia | |
| HP:0025337 | Red eye | |
| HP:0007663 | Reduced visual acuity | |
| HP:0031190 | Superficial dermal perivascular inflammatory infiltrate | |
| HP:0100585 | Telangiectasia of the skin | |
| Excluded (0%) | ||
| HP:0010473 | Porphyrinuria | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 30585497[PMID]_10642674[PMID]_3342168[PMID]_25209448[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/330058
- This record as JSON: /data/d/330058.json