Classic eosinophilic pustular folliculitis
Synonyms: Classic EPF · Ofuji disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult
- Status
- active
A rare skin disease characterized by non-infectious eosinophilic infiltration of the hair follicles. Patients present with chronically recurrent crops of itchy follicular papulopustules with peripheral extension and central clearing. Small papules tend to enlarge and coalesce into large plaques. The lesions favor the face and trunk, although the extremities can also be involved. Laboratory analysis may show peripheral leukocytosis and eosinophilia, while, clinically, systemic involvement is not evident.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Classic eosinophilic pustular folliculitis | Classic EPF · Ofuji disease |
| fr | Folliculite pustuleuse à éosinophiles classique | Folliculite pustuleuse éosinophilique classique · Maladie d'Ofuji |
| de | Klassische eosinophile pustulöse Follikulitis | Klassische EPF · Ofuji-Syndrom · Sterile eosinophile Pustulose Ofuji |
| es | Foliculitis pustulosa eosinofílica clásica | Enfermedad de Ofuji · FPE clásica |
| it | Follicolite pustolosa eosinofila classica | EPF classica · Malattia di Ofuji |
| nl | Klassieke eosinofiele pustulaire folliculitis | Klassieke EPF · Ziekte van Ofuji |
| pt | Foliculite pustular eosinofílica clássica | Doença Ofuji · EPF clássica |
| pl | Klasyczne eozynofilowe krostkowe zapalenie mieszków włosowych | = en |
| cs | Klasická eozinofilní pustulózní folikulitida | = en |
| uk | Класичний еозинофільний пустульозний фолікуліт | Класичний EPF · Хвороба Офудзі |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L73.8 | NTBT | attributed | validated |
| ICD-11 | ED9Y | NTBT | index | validated |
| MONDO | 0850048 | E | validated | |
| UMLS | C0406305 | E | validated | |
| MedDRA | 10052834 | 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:90077Other acquired 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 | Unknown | — | Worldwide | Class only | Validated | 37983361[PMID]_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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/617408
- This record as JSON: /data/d/617408.json