raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:90000

Erythema elevatum diutinum

Type
Disease
Group
Disorder
Inheritance
Unknown
Age of onset
All ages
Status
active

Erythema elevatum diutinum (EED) is a distinctive form of chronic cutaneous vasculitis, belonging to the group of the neutrophilic dermatoses.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enErythema elevatum diutinum
frErythema elevatum diutinum
deErythema elevatum diutinum
esEritema elevatum diutinum
itEritema elevatum diutinum
nlErythema elevatum diutinum
ptEritema elevado diutino
plRumień wyniosły i długotrwały
csErythema elevatum diutinum
ukЕритема підвищеної чутливості (Erythema elevatum diutinum)
zh持久性隆起性红斑
trEritema elevatum diutinum

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10L95.1Especificvalidated
ICD-11EF40.2YNTBTindexvalidated
MONDO0019526Evalidated
MeSHC535509Evalidated
UMLSC0263398Evalidated
GARD8653Evalidated
MedDRA10056968Evalidated

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

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

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

8 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0002829Arthralgia
HP:0000988Skin rash
HP:0200029Vasculitis in the skin
Frequent (79-30%)
HP:0008066Abnormal blistering of the skin
HP:0010702Increased circulating immunoglobulin concentration
HP:0200037Skin vesicle
Occasional (29-5%)
HP:0003326Myalgia
HP:0200036Skin nodule

Run the differential on the 3 obligate / very frequent terms

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Sources for this card