Nonpharmacologic treatments such as oil baths and emollients should also be considered. If the condition appears classic for PUPPP, it can be managed symptomatically. However, if there is any question about the diagnosis, referral to a dermatologist is prudent.
Sequelae. No increase in maternal or fetal morbidity or mortality is associated with PUPPP. Recurrence is fairly uncommon, as the disease primarily affects women during their first pregnancy.
FIGURE 2
Pruritic urticarial papules and plaques of pregnancy
Intrahepatic cholestasis of pregnancy
This condition is also called recurrent or idiopathic jaundice of pregnancy, obstetric cholestasis, and pruritus gravidarum. ICP is caused by disruption of hepatic bile flow during pregnancy. It has been recorded at a rate of approximately 10 to 150 per 10,000 pregnancies in Europe and 70 per 10,000 in the United States.12 In 80% of patients, the time of onset is after the 30th week.14 Although this disorder is not primarily a dermatosis of pregnancy, it is a pruritic condition that often presents with excoriations in pregnant women and is associated with fetal morbidity and mortality. It’s important to be able to identify this disease early to minimize sequelae.
Presentation. There are no primary lesions with ICP. The primary presenting symptom is a generalized pruritus affecting the palms and soles, and sometimes extending to the legs and abdomen (FIGURE 3). This itching is often so severe that it leads to chronic insomnia. You may see secondary skin lesions, such as erythema and excoriations. Observable jaundice occurs in 10% to 20% of patients.3 These patients do not develop the encephalopathy that is associated with cholestasis in the nonpregnant state, however.14
Pathophysiology. The genesis of this condition is thought to be a combination of genetic and environmental factors. A family history of the disorder is present in half the cases, and cases with a familial component tend to be more severe.15 ICP may be an exaggerated response to increased estrogen levels in pregnancy, but the mechanism of this response is unknown.16
Differential. Other conditions that must be considered in making the diagnosis are viral hepatitis, gallbladder disease, PG, PUPPP, drug hepatotoxicity, primary biliary cirrhosis, and uremia.
Diagnosis. Laboratory values are the definitive diagnostic tool in this condition. Increased serum bile acids are the single most sensitive test. Average levels of serum bile acids in pregnancy are 6.6 mcmol/L, with an upper limit of 11. The average value in women with ICP is 47 mcmol/L.17
While serum acids remain the gold standard, a recent study showed elevated urine bile acids to have 100% sensitivity and 83% specificity for ICP.18 In 55% to 60% of cases, the liver enzymes aspartate aminotransferase (AST) and alanine aminotransferase (ALT) are mildly increased. Steatorrhea is often noted by the patient, and is followed by vitamin K deficiency.17
Treatment. The current standard of care for ICP is treatment with ursodeoxycholic acid (UDCA). In 4 controlled trials, UDCA showed a sustained decrease in serum bile acids.19-22 Doses used in these trials have varied between 450 and 1200 mg per day.
Before UDCA treatment was available, the disorder was treated with cholestyramine, which could bring about a 70% rate of response. The drawback to cholestyramine treatment is that it precipitates vitamin K, which is already compromised by the disease process. Further, its onset of action is slow.3
Elective delivery is indicated for ICP, particularly in patients with significant clinical presentations.12 Delivery for ICP should be performed around week 37 to 38, as stillbirths tend to cluster around weeks 37 to 39.14 Given the significant fetal mortality associated with this condition (see below), ICP should be managed by a clinician experienced with the disease, likely a gastroenterologist.
Sequelae. The impact of this maternal disorder on the fetus can be disastrous: a 10% to 15% rate of perinatal death, and a 30% to 40% rate of premature labor have been seen with ICP.14 Fortunately, rates of preterm labor are strongly correlated with levels of bile acids, so that as bile acid levels are reduced with UDCA treatment, rates of preterm labor also go down. Currently, management of the condition has reduced rates of perinatal death to 3.5%. There is no evidence of fetal growth retardation.14
FIGURE 3
Intrahepatic cholestasis of pregnancy
Dermatoses triggered by pregnancy
Eczema of pregnancy/prurigo of pregnancy
Eczema of pregnancy/prurigo of pregnancy (EP/PP) may not actually be correlated with the pregnant state. Both conditions manifest as eczematous lesions in an atopic distribution. Although they have been described in the literature as separate entities, the lack of clinical distinction between them led Ambros-Rudolph and colleagues to combine them under the umbrella term, atopic eruption of pregnancy.23 In some patients, at least, they may be preexisting conditions that pregnancy exacerbates. One study of 255 patients with the condition found that 20% had had the lesions before they became pregnant.23 However, the tendency of the condition to be markedly worsened by pregnancy leads us to include it here.