raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:535

Rare cutaneous lupus erythematosus

Type
Clinical group
Group
Group of disorders
Inheritance
Multigenic/multifactorial
Age of onset
All ages
Status
active

Rare cutaneous lupus erythematosus (CLE) is an autoimmune disease that denotes a heterogeneous spectrum of clinical manifestations affecting the skin and can be divided into 4 categories: acute CLE (ACLE); subacute CLE (SCLE); chronic CLE (CCLE; the most diverse form); and intermittent CLE (ICLE). CLE can either occur alone or associated with systemic lupus erythematosus (SLE).

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enRare cutaneous lupus erythematosus
frLupus érythémateux cutané rare
deLupus erythematodes, kutaner seltener
esLupus eritematoso cutáneo raro
itLupus eritematoso cutaneo
nlZeldzame cutane lupus erythematosus
ptLúpus eritematoso cutâneo raro
plRzadka postać tocznia rumieniowatego skórnego
csKožní lupus erythematodes
ukРідкісний шкірний червоний вовчак
zh罕见皮肤红斑狼疮
trNadir kutanöz lupus eritematozus

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO18887Evalidated
UMLSC5680424Evalidated
GARD6225Evalidated
MedDRA10056509Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

Children (2):

Codes that Orphanet moved or referred to this one:

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

4 records
TypeClassValueAreaQualificationValidationSource
Point prevalence1-5 / 10 00050 / 100 000EuropeValue and classValidatedEuropean Medicines Agency 2007[OTHER]
Annual incidence1-9 / 100 0004.3 / 100 000United StatesValue and classValidated19289752[PMID]
Point prevalence6-9 / 10 00073 / 100 000United StatesValue and classValidated19289752[PMID]
Annual incidence1-9 / 100 0004 / 100 000SwedenValue and classValidated21574972[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

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Sources for this card