raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:33408

Bullous lichen planus

Type
Disease
Group
Disorder
Inheritance
Autosomal dominant, Not applicable
Age of onset
Childhood
Status
active

Bullous lichen planus is a variant of rare lichen planus characterized by the development of vesico-bullous lesions.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enBullous lichen planus
frLichen bulleux
deLichen bullosusLichen planus bullosus
esLiquen plano ampollar
itLichen planus bolloso
nlBulleuze lichen planus
ptLíquen plano bolhoso
plLiszaj płaski pęcherzowy
csBulózní lichen planus
ukБульозний плоский лишай
zh大疱性扁平苔癣
trBüllöz liken planus

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10L43.1Especificvalidated
ICD-11EA91.YNTBTindexvalidated
MONDO0018056Evalidated
UMLSC0023648Evalidated
MedDRA10056960Evalidated

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 prevalenceUnknown—WorldwideClass onlyNot 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

5 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0008066Abnormal blistering of the skin
HP:0100783Breast aplasia
HP:0100725Lichenification
HP:0200034Papule
Occasional (29-5%)
HP:0000989Pruritus

Run the differential on the 4 obligate / very frequent terms

7

Sources for this card