Pruritic urticarial papules and plaques of pregnancy

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ORPHA:64745OMIM:178995O26.8
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Overview

Pruritic urticarial papules and plaques of pregnancy (PUPPP), also known as polymorphic eruption of pregnancy (PEP), is a benign, self-limiting skin condition that occurs most commonly during the third trimester of pregnancy, particularly in first pregnancies (primigravidae). It is the most common specific dermatosis of pregnancy. The condition is characterized by intensely itchy (pruritic) red papules and urticarial plaques that typically begin within the abdominal striae (stretch marks), often sparing the periumbilical area. The rash may subsequently spread to the thighs, buttocks, and occasionally the arms, but rarely involves the face, palms, or soles. The skin is the primary organ system affected, and importantly, PUPPP poses no risk to the mother or fetus beyond the significant discomfort caused by the intense itching. The exact cause of PUPPP remains unclear, but it has been associated with rapid abdominal distension, multiple gestations (twins or higher-order pregnancies), and excessive maternal weight gain. It is more frequently observed in women carrying male fetuses. The condition typically resolves spontaneously within days to weeks after delivery and rarely recurs in subsequent pregnancies. Treatment is symptomatic and aimed at relieving pruritus. First-line therapies include topical corticosteroids (medium to high potency), emollients, and oral antihistamines. In severe cases, a short course of systemic corticosteroids may be considered. Cool baths and moisturizers can also provide symptomatic relief. No long-term complications for mother or baby have been documented.

Also known as:

Clinical phenotype terms— hover any for plain English:

Urticarial plaqueHP:0030351ParakeratosisHP:0001036Increased body weightHP:0004324Pruritis on abdomenHP:0030898Skin vesicleHP:0200037Facial erythemaHP:0001041Pruritis on handHP:0030899Pruritus on footHP:0030900Palmar pruritusHP:0031248Abnormal dermoepidermal junction morphologyHP:0031538Abnormality of the plantar skin of footHP:0100872Palmoplantar erythemaHP:0025493Pruritis on breastHP:0030901Linear IgG deposits along the epidermal basement membrane zoneHP:0031540
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Pruritic urticarial papules and plaques of pregnancy.

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No specialists are currently listed for Pruritic urticarial papules and plaques of pregnancy.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Pruritic urticarial papules and plaques of pregnancy.

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Common questions about Pruritic urticarial papules and plaques of pregnancy

What is Pruritic urticarial papules and plaques of pregnancy?

Pruritic urticarial papules and plaques of pregnancy (PUPPP), also known as polymorphic eruption of pregnancy (PEP), is a benign, self-limiting skin condition that occurs most commonly during the third trimester of pregnancy, particularly in first pregnancies (primigravidae). It is the most common specific dermatosis of pregnancy. The condition is characterized by intensely itchy (pruritic) red papules and urticarial plaques that typically begin within the abdominal striae (stretch marks), often sparing the periumbilical area. The rash may subsequently spread to the thighs, buttocks, and occas

How is Pruritic urticarial papules and plaques of pregnancy inherited?

Pruritic urticarial papules and plaques of pregnancy follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Pruritic urticarial papules and plaques of pregnancy typically begin?

Typical onset of Pruritic urticarial papules and plaques of pregnancy is adult. Age of onset can vary across affected individuals.