raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:64745

Pruritic urticarial papules and plaques of pregnancy

Synonyms: PUPPP · Polymorphic eruption of pregnancy

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

A rare skin disease characterized by urticarial papules and plaques with severe pruritus mainly on the abdomen, buttocks, and proximal thighs. The condition usually develops during the third trimester of the first pregnancy, although presentation in the postpartum period, which may also feature other types of skin lesions, has been described in some cases. The symptoms generally resolve within few weeks.

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Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPruritic urticarial papules and plaques of pregnancyPUPPP · Polymorphic eruption of pregnancy
frPlaques et papules prurigineuses urticariennes de la grossesseEruption polymorphe de la grossesse · PUPPP
dePruritische urtikarielle Papeln und Plaques in der SchwangerschaftPUPPP · Schwangerschaftsdermatose, polymorphe
esPápulas urticariales pruriginosas y placas de embarazoErupción polimórfica del embarazo
itPapule e placche orticarioidi pruriginose della gravidanzaEruzione polimorfa della gravidanza
nlPruritische urticariële papels en plaques bij zwangerschapPUPPP · Polymorfe eruptie bij zwangerschap
ptPápulas e placas pruriginosa urticárias da gravidezErupção polimórfica da gravidez
plSwędzące grudki i ogniska pokrzywkowe w przebiegu ciążyWielopostaciowe osutki ciążowe
csPruritické urtikariální papuly a plaky v graviditěPolymorfní erupce v těhotenství
ukСверблячі кропивяні папули та бляшки при вагітностіПоліморфні висипання під час вагітності · PUPPP
zh妊娠瘙痒性荨麻疹性丘疹及斑块妊娠性多形疹
trGebeliğin döküntülü kaşıntısıPUPPP · Gebelikte polimorfik patlama

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
OMIM178995Evalidated
ICD-10O26.8NTBTattributedvalidated
ICD-11JA65.12Especificvalidated
MONDO0008353Evalidated
MeSHC535817Evalidated
UMLSC0269680Evalidated
GARD9635Evalidated
MedDRA10066100Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET_27960565[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.

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HPO phenotypes

21 annotations · validated 2024-07-09
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0030350Erythematous papule
HP:0000989Pruritus
HP:0001065Striae distensae
HP:0030351Urticarial plaque
Frequent (79-30%)
HP:0025474Erythematous plaque
HP:0004324Increased body weight
HP:0001036Parakeratosis
HP:0030898Pruritis on abdomen
HP:0200037Skin vesicle
Occasional (29-5%)
HP:0031538Abnormal dermoepidermal junction morphology
HP:0100872Abnormality of the plantar skin of foot
HP:0001041Facial erythema
HP:0031248Palmar pruritus
HP:0030899Pruritis on hand
HP:0030900Pruritus on foot
Very rare (<4-1%)
HP:0008066Abnormal blistering of the skin
HP:0000964Eczematoid dermatitis
HP:0025493Palmoplantar erythema
HP:0030901Pruritis on breast
Excluded (0%)
HP:0031541Linear C3 deposits along the epidermal basement membrane zone
HP:0031540Linear IgG deposits along the epidermal basement membrane zone

Run the differential on the 4 obligate / very frequent terms

Annotation source: 9843004[PMID]_30969522[PMID]_22909357[PMID]_31903969[PMID]

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Sources for this card