Dermatology

Pruritic Urticarial Papules and Plaques of Pregnancy (polymorphic eruption of pregnancy, toxemic erythema of pregnancy, and late prurigo of pregnancy)

Pruritic Urticarial Papules and Plaques of Pregnancy (PUPPP), polymorphic eruption of pregnancy, toxemic erythema of pregnancy, and late prurigo of pregnancy, ICD-9-CM 646.8

Are You Confident of the Diagnosis?

Pruriticurticarial papules and plaques of pregnancy (PUPPP) tend to occur during the later third trimester or the immediate postpartum period. Rarely, they may be seen any time after the first trimester. PUPPP tends to be more common in primiparous women, women with large-for-date babies and those patients carrying twins or triplets. The eruption typically begins within the abdominal striae distensae as pruritic urticarial papules that spread to the thighs, buttocks, arms, and legs, with characteristic periumbilical sparing. The face, palms, soles, and mucous membranes tend to be spared. The morphology of the eruption may vary greatly throughout its duration, and patients may develop microvesicles, urticarial wheals, papules, erythematous plaques, target lesions, and scaling.

PUPPP tends to disappear quickly before term or within several days of delivery. It tends not to recur in subsequent pregnancies unless associated with a multiple gestation. Clinical findings tend to vary morphologically, and no diagnostic test exists for disease confirmation. PUPPP is generally considered a diagnosis of exclusion, which depends on a typical clinical presentation, normal laboratory tests, and negative direct immunofluorescence (DIF).

Who is at Risk for Developing this Disease?

PUPPP is second only to eczema as the most common far of the pregnancy dermatoses, with a worldwide incidence of 1:160 pregnancies. It shows no predilection for any particular ethnicity. It tends to be more common in primiparous women, women with large-for-date babies and those patients carrying twins or triplets.

What is the Cause of the Disease?

The cause of PUPPP remains unknown. It has no association with other autoimmune diseases or pre-eclampsia, but does tend to be linked with an increased frequency of increased maternal weight gain, as in women with large-for-gestational age babies and those patients carrying twins or triplets. Therefore, several theories as to the etiology of the disease exist:

--Excessive abdominal distension may result in destruction of elastic fibers, releasing antigenic molecules, causing an inflammatory process. This may explain PUPPP occurring in women with increased weight gain and initial lesions occurring within the striae distensae (Figure 1, Figure 2).

Figure 1.

Erythematous plaques forming within striae of a gravid female characteristic of PUPPP.

Figure 2.

The eruption typically began within the abdominal striae distensae as pruritic urticarial papules that spread to the thighs, buttocks, arms, and legs, with characteristic periumbilical sparing.

--Hormonal factors may play a role in the pathogenesis. One prospective study showed a significantly reduced serum cortisol level compared with normal pregnant controls.

Fetal factors may contribute to the etiology, as it has been reported that a preponderance of male infants, with a male: female ratio of 2:1, are born to mothers with this disease.

Systemic Implications and Complications

There tends to be no increased risk to the mother or fetus. One study noted that 3 cases out of 44 necessitated early delivery of the baby due to severe intractable pruritus, but no adverse effects on the fetuses as a direct result of maternal PUPPP were observed.

Treatment Options

Most patients will benefit from topical corticosteroids and oral antihistamines. Occasionally, severe cases of distressing pruritus will require the use of oral corticosteroids or phototherapy.

Optimal Therapeutic Approach for this Disease

Most patients will benefit from topical corticosteroids and oral antihistamines. Recommned diphenhydramine 25-50mg every 6 hours and nightly as needed as well as clobetasol propionate 0.05% cream twice daily. After 2 weeks of using clobetasol proprionate, then patients should step down to a mid-potent topical steroid such as triamcinolone 0.1% cream twice daily up to 4 additional weeks if itching persists. Conditioning the skin with moisturizers is also helpful.

Occasionally, severe cases of distressing pruritus will require the use of low-dose oral corticosteroids such as prednisone 10 to 20mg daily. Phototherapy with narrowband ultraviolet light two to three times per week can also be helpful.

Patient Management

If the patient’s presentation is classic, then manage the patient symptomatically as there tends to be no increased risk to the mother or fetus. Midpotent topical corticosteroids should relieve pruritus with in 1-2 days. Insomnia secondary to pruritus is common and, therefore, oral antihistamines in conjunction with topical therapy are recommended.

Unusual Clinical Scenarios to Consider in Patient Management

The main concern is differentiating early pemphigoid gestionis from PUPPP. The characteristic periumbilical sparing of PUPPP can be helpful clinically in differentiating the two diseases. However, a biopsy for direct immunofluorescence (DIF) can be performed if needed. DIF will be negative in PUPPP.

What is the Evidence?

Al-Fares, SI, Vaughan Jones, SA, Black, MM. "The specific dermatoses of pregnancy: a re-appraisal". JEADY. vol. 15. 2001. pp. 197-206.

(This article clarifies specific guidelines for diagnosing the dermatoses of pregnancy.)

Holmes, RC, Black, MM. "The specific dermatoses of pregnancy". J Am Acad Dermatol. vol. 8. 1983. pp. 405-12.

(This article that defines the various catergories of skin lesions occurring in pregnancy.)

Charles-Holmes, R. "Polymorphic eruption of pregnancy". Semin Dermatol. vol. 8. 1989. pp. 18-22.

(This article clarifies the range of clinical features of polymorphic eruption of pregnancy.)

Bremmer, M, Driscoll, MS, Colgan, R. "Six skin disorders of pregnancy: a management guide". OBG Management. vol. 22. 2010. pp. 24-33.

(This article is a brief summary for the nondermatologist of the main dermatoses of pregnancy with a user-friendly table that aids in sorting the diagnostic clues and treatment pearls.)

Vaughan Jones, SA, Hern, S, Nelson-Piercy, C, Seed, PT, Black, MM. "A prospective study of 200 women with dermatoses of pregnancy correlating clinical findings with hormonal and immunopathological profiles". Br J Dermatol. vol. 141. 1999. pp. 71-81.

(This article clarifies specific lab and pathology diagnostic clues for diagnosing the dermatoses of pregnancy.)

Shornick, JK, Bolognia, JL, Jorizzo, JL, Rapini, RP. "Pregnancy dermatoses". Mosby. 2003. pp. 425-32.

(This is a comprehenisve and detailed chapter in a leading dermatology textbook.)
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