Subacute cutaneous lupus erythematosus
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult, Elderly
- Status
- active
A form of cutaneous lupus erythematosus (CLE) that can present either as a non-scarring, annular photo-distributed dermatosis or psoriasiform plaques. This disorder is associated with anti-Ro/SSA antibodies and can be drug-induced.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Subacute cutaneous lupus erythematosus | |
| fr | Lupus érythémateux cutané subaigu | |
| de | Lupus erythematodes, kutaner, subakuter | |
| es | Lupus eritematoso cutáneo subagudo | |
| it | Lupus eritematoso cutaneo subacuto | |
| nl | Subacute cutane lupus erythematosus | |
| pt | Lupus eritematoso cutâneo subagudo | |
| pl | Podostry toczeń rumieniowy skórny | |
| cs | Subakutní kožní lupus erythematodes | |
| uk | Підгострий шкірний червоний вовчак | |
| zh | 亚急性皮肤红斑狼疮 | |
| tr | Subakut kutanöz lupus eritematozus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L93.1 | E | specific | validated |
| ICD-11 | EB50 | E | specific | validated |
| MONDO | 0015573 | E | validated | |
| UMLS | C0024140 | E | validated | |
| MedDRA | 10057903 | 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:535Rare cutaneous lupus erythematosus
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 |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.63 / 100 000 | United States | Value and class | Validated | 19289752[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.4805 / 100 000 | United States | Value and class | Validated | 32141953[PMID]_35359921[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
19 annotations · validated 2023-04-11| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0033555 | Anti-Ro/SS-A antibody positivity | |
| Frequent (79-30%) | ||
| HP:0025528 | Annular cutaneous lesion | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0000992 | Cutaneous photosensitivity | |
| HP:0025526 | Psoriasiform lesion | |
| HP:0002923 | Rheumatoid factor positive | |
| Occasional (29-5%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0032235 | Anti-La/SS-B antibody positivity | |
| HP:0020151 | Anti-dsDNA antibody positivity | |
| HP:0033558 | Anti-histone antibody positivity | |
| HP:0003613 | Antiphospholipid antibody positivity | |
| HP:0004334 | Dermal atrophy | |
| HP:0007417 | Discoid lupus rash | |
| HP:0000962 | Hyperkeratosis | |
| HP:0031541 | Linear C3 deposits along the epidermal basement membrane zone | |
| HP:0031540 | Linear IgG deposits along the epidermal basement membrane zone | |
| Very rare (<4-1%) | ||
| HP:0100825 | Cheilitis | |
| HP:0025300 | Malar rash | |
| HP:0200029 | Vasculitis in the skin | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 28185656[PMID]_27799135[PMID]_17207162[PMID]_35907579[PMID]_35198470[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/163525
- This record as JSON: /data/d/163525.json