raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:90397

Self-healing papular mucinosis

Type
Disease
Group
Disorder
Age of onset
All ages
Status
active

Self-healing papular mucinosis is a rare form of localized lichen myxedematosus occurring primarily in children and characterized by the development of mucinous papules on various parts of the body (face, neck, trunk, and limbs) that resolve spontaneously within some weeks to months. Systemic symptoms can be observed such as fever, arthralgias and weakness.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enSelf-healing papular mucinosis
frMucinose cutanée juvénile spontanément régressive
deMuzinose, papulöse, selbstheilende
esMucinosis papular de resolución espontánea
itMucinosi papulosa spontaneamente regressiva
nlZelfhelende papulaire mucinose
ptMucinose papular autolimitada
plSamowyleczalna mucynoza grudkowa
csSelf-healing papulární mucinóza
ukСамовиліковний папульозний муциноз
zh自愈性丘疹性粘蛋白沉积症
trKendi kendini iyileştiren papüler müsinoz

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10L98.5NTBTattributedvalidated
ICD-11EB90.1YNTBTindexvalidated
MONDO0019582Evalidated
UMLSC0406657Evalidated

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 (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families24 casesWorldwideCase(s)ValidatedORPHANET
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidatedORPHANET

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.

7

Sources for this card