raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:700

Alopecia totalis

Type
Disease
Group
Disorder
Inheritance
Multigenic/multifactorial
Age of onset
All ages
Status
active

A form of alopecia areata, an inflammatory disease of the hair follicle, characterized by a complete loss of hair of the entire scalp which becomes glabrous.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAlopecia totalis
frPelade totale
deAlopecia totalis
esAlopecia total
itAlopecia areata totale
nlAlopecia totalis
ptAlopecia totalis
plŁysienie całkowite
csAlopecia totalis
ukТотальна алопеція
zh全秃
trAlopesi totalis

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
OMIM104000NTBTvalidated
OMIM300042BTNTvalidated
OMIM610753BTNTvalidated
ICD-10L63.0Especificvalidated
ICD-11ED70.21Especificvalidated
MONDO0019080Evalidated
UMLSC0263504Evalidated
GARD1470Evalidated
MedDRA10001766Evalidated

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

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalence1-5 / 10 00010.5 / 100 000EuropeValue and classNot 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

10 annotations · validated 2023-09-18
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0002293Alopecia of scalp
HP:0007418Alopecia totalis
Occasional (29-5%)
HP:0002960Autoimmunity
HP:0001808Fragile nails
HP:0002037Inflammation of the large intestine
HP:0001803Nail pits
HP:0001806Onycholysis
HP:0030804Trachyonychia
HP:0100651Type I diabetes mellitus
HP:0001045Vitiligo

Run the differential on the 2 obligate / very frequent terms

Annotation source: 33085372[PMID]

7

Sources for this card