Discoid lupus erythematosus
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare form of chronic cutaneous lupus erythematosus characterized by erythematous, scaly papules and plaques preferentially occurring on sun-exposed skin areas (scalp, face, and ears) and exhibiting follicular plugging, pigmentary changes, and central atrophy, scarring, and telangiectasia. Skin biopsy shows a perivascular and periadnexal lymphocytic infiltrate and involvement of the dermoepidermal junction with thickening of the basement membrane and vacuolar degeneration of the basal cells. A small percentage of patients may develop systemic lupus erythematosus.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Discoid lupus erythematosus | |
| fr | Lupus érythémateux discoïde | |
| de | Lupus erythematodes, diskoider | |
| es | Lupus eritematoso discoide | |
| it | Lupus discoide eritematoso | |
| nl | Discoïde lupus erythematosus | |
| pt | Lúpus eritematoso discóide | |
| pl | Dyskoidalny toczeń rumieniowaty | |
| cs | Diskoidní lupus erythematodes | |
| uk | Дискоїдний червоний вовчак | |
| zh | 盘状红斑狼疮 | |
| tr | Diskoid lupus eritematozus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L93.0 | E | specific | validated |
| ICD-11 | EB51.0 | E | specific | validated |
| MONDO | 0019558 | E | validated | |
| MeSH | D008179 | E | validated | |
| UMLS | C5574816 | E | validated | |
| MedDRA | 10013072 | 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:163531Chronic cutaneous lupus erythematosus
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 3.56 / 100 000 | United States | Value and class | Validated | 19289752[PMID] |
| Annual incidence | 1-9 / 100 000 | 3.2 / 100 000 | Sweden | Value and class | Validated | 21574972[PMID] |
| Point prevalence | 1-5 / 10 000 | 30 / 100 000 | Worldwide | Value and class | 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
10 annotations · validated 2024-09-26| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0001075 | Atrophic scars | |
| HP:0030350 | Erythematous papule | |
| HP:0025474 | Erythematous plaque | |
| HP:0007502 | Follicular hyperkeratosis | |
| HP:0001010 | Hypopigmentation of the skin | |
| HP:4000107 | Positive lupus band test | |
| HP:0004552 | Scarring alopecia of scalp | |
| HP:0100585 | Telangiectasia of the skin | |
| Occasional (29-5%) | ||
| HP:0002829 | Arthralgia | |
| Very rare (<4-1%) | ||
| HP:0006739 | Squamous cell carcinoma of the skin | |
Annotation source: 29630197[PMID]_28606712[PMID]_39166468[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90281
- This record as JSON: /data/d/90281.json