Pruritic urticarial papules plaques of pregnancy

Alternate names

Polymorphic eruption of pregnancy; PUPPP; Pruritic urticarial papules and plaques of pregnancy, familial (subtype)

Definition

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.

• Front view of abdomen

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  Side view of abdomen

• Feet and ankle view

Epidemiology

The reported incidence of pruritic urticarial papules and plaques of pregnancy in the literature is 0,5% in single pregnancies, 2.9 to 16% in twin pregnancies and 14 to 17 % in triplet pregnancies. It is seen predominantly in primigravidas women and tends not to relapse in later pregnancies. Reports have linked excessive maternal weight gain and increased newborn birth weight in association with PUPPP. Patients affected were interestingly Rh-positive in a study of Ghazeeri and colleagues, and in 89% of them, conception was the product of in vitro fertilization.

Cause

The cause of pruritic urticarial papules and plaques of pregnancy is still unknown, although various etiological theories have been proposed, including abdominal distension, hormonal changes, placental factors and the role of fetal DNA in skin lesions of patients with PUPPP.

Signs and symptoms

Pruritic urticarial papules and plaques of pregnancy occur most often during the last month of pregnancy and only rarely appear in the postpartum period. The rash consists of itchy small erythematous and edematous papules and plaques usually first start in the stretch marks, typically with periumbili­cal sparing. The lesions can coalesce to form larger urticarial abdominal plaques often surrounded by blanched halos. The eruption spreads over a matter of days, to the trunk and the extremities, but rarely involves the face, palms, or soles. After that, almost all patients develop more polymorphic features as the disease evolves, includ­ing widespread erythema, targetoid lesions, tiny vesicles, and eczema­tous patches. Mucosal involvement is usually not observed. When PUPPP resolves, typically over an average of 4 weeks spontaneously or with delivery, there is no post-inflammatory pigment change or scarring of the skin. The eruption tends to not recur with subsequent pregnancies.

Diagnosis

Usually, routine laboratory tests are within normal limits in patients with PUPPP. Upon consideration of the diagnosis, the degree of discomfort related to pruritus should be evaluated in all cases as the treatment is mainly symptomatic. Additionally, maternal weight and fetus growth should be plotted during the pregnancy course. Generally, PUPPP is not an indication for early delivery.

Treatment

Only symptomatic treatment is usually necessary for patients with PUPPP. The majority of affected ladies achieve satisfactory control with topical corticosteroids and oral antihistamines. Further, sedating first-generation antihistamines appear to be safe in pregnancy and can be used as adjunct therapy to provide relief from pruritus. For severe disease with disturbed sleep leading to exhaustion of the mother, a short course of systemic corticosteroids can be helpful.

General measures, such as cool, soothing baths, frequent application of emollients and light cotton clothing, offers additional symptomatic relief. Recently, intramuscular injection of autologous whole blood has been suggested as an alternative treatment option in a case of post-partum PUPPP, with both subjective and objective improvement of the symptoms.

Prognosis

Apart from the discomfort of a pruritic urticarial eruption, maternal prognosis still unaffected. Early delivery is rarely used for relief of intractable pruritus. There are no fetal morbidities, and neonatal skin is usually not affected. Recurrence of the eruption in subsequent pregnancies is unusual, except for multiple-gestation pregnancies. In this case, PUPPP tends to be less severe than the first episode.

NIH genetic and rare disease info

• NIH info on Pruritic urticarial papules plaques of pregnancy

Pruritic urticarial papules plaques of pregnancy is a rare disease.

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