Drug-induced lupus erythematosus
Synonyms: DILE
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare, systemic disease with skin involvement characterized by the onset of idiopathic lupus erythematosus-like signs and symptoms resulting from continuous drug intake (>1 month), which resolve when treatment is discontinued, in persons with no history of autoimmune disease. Manifestations are variable and may be systemic (e.g. arthralgia, myalgia, fever, fatigue, serositis, pleuritis, pericarditis), subacute cutaneous (incl. photosensitive, non-scarring, annular, polycyclic or papulosquamous lesions, malar erythema, vasculitis, bullous lesions, erythema multiforme-like changes), and/or chronic cutaneous (typically discoid lesions in sun-exposed areas). Procainamide and hydralazine are the drugs most frequently implicated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Drug-induced lupus erythematosus | DILE |
| fr | Lupus érythémateux induit par les médicaments | = en |
| de | Lupus erythematodes, Medikamenteninduzierter | = en |
| es | Lupus eritematoso inducido por fármacos | DILE · Lupus eritematoso inducido por medicamentos |
| it | Lupus eritematoso indotto dai farmaci | = en |
| nl | Medicatiegeïnduceerde lupus erythematosus | = en |
| pt | Lupus eritmatoso induzido por fármacos | |
| pl | Toczeń rumieniowaty indukowany lekami | = en |
| cs | Léky navozený systémový lupus erythematodes | = en |
| uk | Медикаментозний червоний вовчак | = en |
| zh | 药物诱发红斑狼疮 | = en |
| tr | İlaca bağlı lupus eritematoz | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M32.0 | E | specific | validated |
| ICD-11 | 4A40.1 | E | specific | validated |
| MONDO | 0016474 | E | validated | |
| UMLS | C0263591 | E | validated | |
| MedDRA | 10013706 | 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:182222Rare systemic disease
rare systemic and rheumatological diseases - ORPHA:290836Systemic disease with skin involvement
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet 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
23 annotations · validated 2017-07-03| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001903 | Anemia | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0002829 | Arthralgia | |
| HP:0030057 | Autoimmune antibody positivity | |
| HP:0005421 | Decreased circulating complement C3 concentration | |
| HP:0045042 | Decreased circulating complement C4 concentration | |
| HP:0002094 | Dyspnea | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003236 | Elevated circulating creatine kinase concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0000790 | Hematuria | |
| HP:0003138 | Increased blood urea nitrogen | |
| HP:0025343 | Lupus anticoagulant | |
| HP:0025300 | Malar rash | |
| HP:0003326 | Myalgia | |
| HP:0001698 | Pericardial effusion | |
| HP:0001701 | Pericarditis | |
| HP:0000967 | Petechiae | |
| HP:0005184 | Prolonged QTc interval | |
| HP:0001873 | Thrombocytopenia | |
| Frequent (79-30%) | ||
| HP:0001945 | Fever | |
| HP:0045073 | Serositis | |
| Occasional (29-5%) | ||
| HP:0025142 | Constitutional symptom | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 26420637[PMID]_26710505[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/231111
- This record as JSON: /data/d/231111.json