Lupus erythematosus panniculitis
Synonyms: Lupus erythematosus profundus
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy
- Status
- active
A rare form of chronic cutaneous lupus erythematosus characterized by recurrent, indurated, erythematous plaques and subcutaneous nodules with normal overlying epidermis, occurring predominantly on the face, upper arms, trunk, buttocks, and thighs. The lesions can ulcerate and lead to scarring. Histological findings include lobular lymphocytic panniculitis, hyaline fat necrosis, mucin deposition, and calcification. The condition may be associated with discoid or systemic lupus erythematosus.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Lupus erythematosus panniculitis | Lupus erythematosus profundus |
| fr | Panniculite lupique | Lupus érythémateux profond |
| de | Lupus erythematodes Pannikulitis | Lupus erythematodes profundus · Lupus-Pannikulitis |
| es | Lupus eritematoso profundo | Paniculitis lúpica |
| it | Panniculite da lupus eritematoso | Lupus eritematoso profondo |
| nl | Lupus erythematosus panniculitis | = en |
| pt | Lúpus eritematoso paniculite | |
| pl | Toczeń rumieniowaty głęboki | = en |
| cs | Lupus erythematodes panniculitis | Lupus erythematodes profundus |
| uk | Червоний вовчак панікуліт | Глибокий червоний вовчак |
| zh | 红斑狼疮性脂膜炎 | 深部红斑狼疮 |
| tr | Lupus eritematozus panniküliti | Lupus eritematozus profundus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L93.2 | NTBT | inclusion | validated |
| ICD-11 | EB51.Y | NTBT | index | validated |
| MONDO | 0019561 | E | validated | |
| MeSH | D015435 | E | validated | |
| UMLS | C0030327 | 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.07 / 100 000 | United States | Value and class | Validated | 19289752[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.315 / 100 000 | United States | Value and class | Validated | 9918242[PMID]_19289752[PMID]_30658703[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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90285
- This record as JSON: /data/d/90285.json