raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:2221

Acquired hypertrichosis lanuginosa

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adult
Status
active

A rare cutaneous paraneoplastic disease characterized by the presence of excessive lanugo-type hair on the glabrous skin of face, neck, trunk and limbs that can be associated with additional clinical features such as burning glossitis, papillary hypertrophy of the tongue, diarrhea, dysgeusia, and/or weight loss. It is associated with lymphoma or cancer of the gastrointestinal system, urinary tract, lung, breast, uterus or ovary.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAcquired hypertrichosis lanuginosa
frHypertrichose lanugineuse acquise
deHypertrichosis lanuginosa, erworbene
esHipertricosis lanuginosa adquirida
itIpertricosi lanuginosa acquisita
nlVerworven hypertrichosis lanuginosa
ptHipertricose lanuginosa adquirida
plNabyte nadmierne owłosienie typu płodowego
csZískaná lanuginózní hypertrichóza
ukНабутий гіпертрихоз ланугіноз
zh获得性胎毛增多症
trEdinsel hipertrikoz lanuginoza

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10L68.1Especificvalidated
ICD-11EL10NTBTindexvalidated
MONDO0016380Evalidated
UMLSC0343072Evalidated
GARD2864Evalidated

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/families60 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

18 annotations · validated 2017-09-27
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0000534Abnormal eyebrow morphology
HP:0000492Abnormal eyelid morphology
HP:0002213Fine hair
HP:0002230Generalized hirsutism
HP:0005599Hypopigmentation of hair
HP:0002664Neoplasm
Frequent (79-30%)
HP:0000206Glossitis
HP:0000158Macroglossia
Occasional (29-5%)
HP:0000956Acanthosis nigricans
HP:0002028Chronic diarrhea
HP:0008064Ichthyosis
HP:0002716Lymphadenopathy
HP:0100013Neoplasm of the breast
HP:0100606Neoplasm of the respiratory system
HP:0100615Ovarian neoplasm
HP:0004396Poor appetite
HP:0001072Thickened skin
HP:0001824Weight loss

Run the differential on the 6 obligate / very frequent terms

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Sources for this card