raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:492

Proliferating trichilemmal cyst

Type
Disease
Group
Disorder
Age of onset
Adult
Status
active

A rare large, multinodular, usually benign, tumor that is generally located in the posterior part of the scalp in aged women (over 50 years). It first appears as a painless nodule that later grows into a solid or partially cystic tumor that is mobile over the underlying subcutaneous tissues. It can present ulceration, inflammation or even bleeding and can cause necrosis of the adjacent tissues.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enProliferating trichilemmal cyst
frKyste trichilemmal proliférant
deTrichilemmalzyste, proliferierende
esQuiste triquilemal proliferante
itCisti trichilemmale proliferativa
nlProlifererende trichilemmale cyste
ptQuisto triquilémico proliferativo
plTorbiel włosowa proliferująca
csProliferující trichilemální cysta
ukПроліферуюча трихілемальна кіста
zh增生性毛根鞘囊肿
trÇoğalan trikilemmal kist

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10L72.1NTBTattributedvalidated
ICD-11EK70.1Especificvalidated
ICD-11XH7WJ7Especificvalidated
MONDO0018850Evalidated
UMLSC2959585Evalidated
GARD4509Evalidated

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

0 records

No prevalence record in this release.

6

HPO phenotypes

3 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0200040Epidermoid cyst
HP:0200042Skin ulcer
Frequent (79-30%)
HP:0002209Sparse scalp hair

Run the differential on the 2 obligate / very frequent terms

7

Sources for this card