Atypical lichen myxedematosus
Synonyms: Intermediate lichen myxedematosus
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
An intermediate form of lichen myxedematosus (LM) (a form of mucin dermal deposit) which does not meet the criteria for either scleromyxedema or the localized form. Three clinical subtypes have been described and include scleromyxedema without monoclonal gammopathy; localized forms with monoclonal gammopathy and/or systemic symptoms; localized forms with mixed features of the 5 subtypes of localized LM (discrete form, acral persistent papular mucinosis, self-healing papular mucinosis, papular mucinosis of infancy, and a pure nodular form). The course of atypical LM is unpredictable because only a few cases have been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Atypical lichen myxedematosus | Intermediate lichen myxedematosus |
| fr | Lichen myxoedémateux atypique | Lichen myxoedémateux intermédiaire |
| de | Lichen myxoedematosus, atypischer | Intermediärer Lichen myxödematosus |
| es | Liquen mixedematoso atípico | Liquen mixedematoso intermedio |
| it | Lichen mixedematoso atipico | Lichen mixedematoso intermedio |
| nl | Atypische lichen myxoedematosus | Intermediaire lichen myxoedematosus |
| pt | Líquen mixedematoso, formas atípicas | |
| pl | Atypowy liszaj śluzowaty | Liszaj śluzowaty średniozaawanowany |
| cs | Atypický lichen myxedematosus | Středně pokročilý lichen myxedematosus |
| uk | Атиповий мікседематозний ліхен | Проміжний лишай мікседематозний |
| zh | 非典型粘液水肿性苔癣 | 中间型黏液水肿性苔藓 |
| tr | Atipik liken miksödemöz | Orta dereceli liken miksödemöz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L98.5 | NTBT | attributed | validated |
| ICD-11 | EB90.11 | NTBT | attributed | validated |
| MONDO | 0019447 | E | validated | |
| UMLS | C4510874 | 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:402007Lichen myxedematosus
rare skin diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 20 cases | Worldwide | Case(s) | Validated | [EXPERT] | |
| 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86797
- This record as JSON: /data/d/86797.json