raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:281241

Autosomal ichthyosis syndrome with fatal disease course

Type
Category
Group
Group of disorders
Status
active

This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAutosomal ichthyosis syndrome with fatal disease course
frIchtyose syndromique autosomique avec évolution fatale de la maladie
deIchthyose-Syndrom, autosmales, mit fatalem Krankheitsverlauf
esSíndrome de ictiosis autosómica con enfermedad de curso fatal
itIttiosi sindromica autosomica con decorso fatale della malattia
nlAutosomaal ichthyosis-syndroom met fataal ziekteverloop
ptSíndrome ictiose autossómica com doença de curso fatal
plAutosomalny zespół rybiej łuski ze śmiertelnym przebiegiem choroby
csAutozomálně dědičné syndromy s ichtyózou a fatálním průběhem
ukСиндром аутосомного іхтіозу з фатальним перебігом захворювання
zh伴有致命性病程的常染色体遗传鱼鳞病综合征
trFatal hastalık seyri ile seyredenotozomal iktiyoz sendromu
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO17273Evalidated
UMLSC5681026Evalidated

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):

Children (5):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

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

7

Sources for this card