raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:63275

Pemphigoid gestationis

Synonyms: Gestational pemphigoid

Type
Disease
Group
Disorder
Age of onset
Adolescent, Adult
Status
active

A rare autoimmune bullous skin disease characterized by pruritus with or without polymorphic skin eruption, affecting pregnant women typically during the second and third trimester.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPemphigoid gestationisGestational pemphigoid
frPemphigoïde gravidiquePemphigoïde de la grossesse · Pemphigoïde gestationnelle
dePemphigoid gestationisSchwangerschafts-Pemphigoid
esPenfigoide gestationisHerpes gestationis · Penfigoide gestacional
itPemfigoide gestazionalePemfigoide gravidico
nlPemphigoid gestationisGestationeel pemfigoïd
ptPenfigoide gestacional
plPemfigoid ciężarnych= en
csGestační pemphigoidGestační pemfigoid
ukГестаційний пемфігоїдГестаційний пемфігоїд
zh妊娠性类天疱疮妊娠性类天疱疮
trPemfigoid gestationisPemfigoid gestasyones

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10O26.4NTBTattributedvalidated
ICD-11JA65.10Especificvalidated
MONDO0006558Evalidated
MeSHD006559Evalidated
UMLSC0019343Evalidated
GARD6497Evalidated
MedDRA10019939Evalidated

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

4 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 1 000 0000.2 / 100 000GermanyValue and classValidated19170813[PMID]
Annual incidence<1 / 1 000 0000.05 / 100 000FranceValue and classValidated7826096[PMID]_23237497[PMID]
Annual incidence1-9 / 1 000 0000.18 / 100 000KuwaitValue and classValidated15569006[PMID]
Point prevalence1-9 / 1 000 0000.693 / 100 000GermanyValue and classValidated27456755[PMID]

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

6 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0008066Abnormal blistering of the skin
HP:0001622Premature birth
HP:0000989Pruritus
HP:0200037Skin vesicle
Frequent (79-30%)
HP:0001508Failure to thrive
HP:0001511Intrauterine growth retardation

Run the differential on the 4 obligate / very frequent terms

Annotation source: 25178359[PMID]

7

Sources for this card