Linear atrophoderma of Moulin
- Type
- Disease
- Group
- Disorder
- Age of onset
- Childhood
- Status
- active
Linear atrophoderma of Moulin (LAM) is characterized by mildly atrophic and hyperpigmented band-like lesions that follow the lines of Blaschko on the trunk or limbs. Since its initial description in 1992, less than 30 cases have been reported in the literature. Onset occurs during childhood or adolescence and the disease is non-progressive. There is no prior inflammation or subsequent scleroderma. The aetiology is unknown but as LAM follows the lines of Blaschko it has been suggested that the disease is caused by mosaicism of a predisposing gene.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Linear atrophoderma of Moulin | |
| fr | Atrophodermie linéaire de Moulin | |
| de | Atrophodermie, lineare, Typ Moulin | Atrophodermia linearis Moulin |
| es | Atrofodermia lineal de Moulin | |
| it | Atrofoderma lineare di Moulin | |
| nl | Lineaire atrofodermie van Moulin | |
| pt | Atrofodermia linear de Moulin | |
| pl | Linijna atrofodermia Moulina | |
| cs | Lineární Moulinova atrophoderma | |
| uk | Лінійна атрофодермія Мулена | |
| zh | Moulin 线状皮肤萎缩 | |
| tr | Lineer Moulin atrofoderması |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L90.8 | NTBT | attributed | validated |
| ICD-11 | EB61.Y | NTBT | index | validated |
| MONDO | 0015371 | E | validated | |
| UMLS | C1274753 | E | validated | |
| GARD | 19932 | 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 (2):
- ORPHA:79381Other dermis disorder
rare skin diseases - ORPHA:477808Other genetic dermis disorder
rare genetic 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 | 30 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.
HPO phenotypes
5 annotations · validated 2016-10-12| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0007546 | Linear hyperpigmentation | Diagnostic criterion |
| Excluded (0%) | ||
| HP:0011123 | Inflammatory abnormality of the skin | Diagnostic criterion |
| HP:0000989 | Pruritus | Diagnostic criterion |
| HP:0100324 | Scleroderma | Diagnostic criterion |
| HP:0030053 | Stiff skin | Diagnostic criterion |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 27051814[PMID]_23989408[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/140933
- This record as JSON: /data/d/140933.json