ORPHA:90396
Acral persistent papular mucinosis
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare chronic form of localized lichen myxedematosus characterized by the development of multiple symmetrical skin-colored mucinous papules exclusively on the extensor surface of the hands and distal forearms.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acral persistent papular mucinosis | |
| fr | Mucinose papuleuse acrale persistante | |
| de | Muzinose, papulöse, akral-persistierende | |
| es | Mucinosis papular acra persistente | |
| it | Mucinosi papulosa acrale persistente | |
| nl | Acrale persisterende papulaire mucinose | |
| pt | Mucinose papular acral persistente | |
| pl | Obwodowa przewlekła mucynoza grudkowa | |
| cs | Akrální perzistentní papulární mucinóza | |
| uk | Акральний персистуючий папульозний муциноз | |
| zh | 肢端持续性丘疹性黏蛋白病 | |
| tr | Akral kalıcı papüler müsinoz |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L98.5 | NTBT | attributed | validated |
| ICD-11 | EB90.1Y | NTBT | index | validated |
| MONDO | 0019581 | E | validated | |
| UMLS | C0406660 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:86795Localized lichen myxedematosus
rare skin diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 40 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | 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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90396
- This record as JSON: /data/d/90396.json