Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Age of onset
- Adult, Elderly
- Status
- active
Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms is a form of atypical lichen myxedematosus, characterized by the appearance of several 2-4 mm erythematous waxy papules confined to a few sites that may be associated with either an immunoglobulin A (IgA) nephropathy in patients with acral persistent papular mucinosis; discrete papular lichen myxedematosus; a scleromyxedema-like involvement, with dysphagia, hoarseness, pulmonary involvement, and carpal tunnel syndrome; myositis without skin sclerosis; or paraproteinemia.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms | |
| fr | Lichen myxoedémateux localisé avec gammopathie monoclonale ou symptômes systémiques | |
| de | Lichen myxoedematosus, lokalisierter, mit monoklonaler Gammopathie/oder systemischen Symptomen | |
| es | Liquen mixedematoso localizado con gammapatía monoclonal o síntomas sistémicos | |
| it | Lichen mixedematoso localizzato con gammapatia monoclonale e/o sintomi sistemici | |
| nl | Gelokaliseerde lichen myxoedematosus met monoklonale gammopathie of systemische symptomen | |
| pt | Líquen mixedematoso localizado com gamopatia monoclonal ou sintomas sistémicos | |
| pl | Zlokalizowany liszaj śluzowaty z gammopatią monoklonalną lub objawami ogólnymi | |
| cs | Lokalizovaný lichen myxedematosus s monoklonální gamapatií nebo systémovými symptomy | |
| uk | Локалізований мікседематозний ліхен з моноклональною гамопатією або системними симптомами | |
| zh | 局限性粘液水肿性苔癣伴单克隆丙种球蛋白病或全身症状 | |
| tr | Monoklonal gammopati veya sistemik semptomlar ile seyreden lokalize liken miksödematozus |
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 | 0019584 | E | validated | |
| UMLS | C5681466 | 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:86797Atypical lichen myxedematosus
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 |
|---|---|---|---|---|---|---|
| Cases/families | 5 cases | Worldwide | Case(s) | Validated | 22235972[PMID] | |
| 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/90399
- This record as JSON: /data/d/90399.json