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.
3. Intrahepatic cholestasis of pregnancy
This condition is also called recurrent or idiopathic jaundice of pregnancy, obstetric cholestasis, and pruritus gravidarum. Intrahepatic cholestasis of pregnancy (ICP) is caused by disruption of hepatic bile flow during pregnancy. It has been recorded at a rate of approximately 10 to 150 of every 10,000 pregnancies in Europe and 70 of every 10,000 in the United States.12 In 80% of patients, 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
FIGURE 3 Intrahepatic cholestasis of pregnancy
ICP lacks primary lesions. Shown here are the secondary erythema and excoriations that results from scratching the intense pruritis.
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 one half of cases; 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 diagnosis. 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 levels of serum bile acids are the single most sensitive test. Average levels of serum bile acids in pregnancy are 6.6 µmol/L, with an upper limit of 11 µmol/L. The average value in women who have ICP is 47 µmol/L.17
Although serum bile acids remain the gold standard, a recent study showed that elevated urine bile acids 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 four controlled trials, UDCA caused a sustained decrease in serum bile acids.19-22 The dosage used in these trials ranged from 450 to 1,200 mg/d.
Before UDCA treatment was available, ICP 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, the onset of action of cholestyramine is slow.3
Elective delivery is indicated for ICP, particularly in patients who have a significant clinical presentation.12 Delivery for ICP should be performed around weeks 37 to 38, as stillbirths tend to cluster around weeks 37 to 39.14 Given the significant fetal mortality associated with ICP (see the next paragraph), the condition 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.14 Fortunately, the rate of preterm labor correlates strongly with the level of bile acids, so that as bile acid levels are reduced with UDCA treatment, the rate of preterm labor also falls. Management of ICP has reduced the rate of perinatal death to 3.5%. No evidence of fetal growth retardation has been noted.14
DERMATOSES TRIGGERED BY PREGNANCY
4. 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, EP/PP may be preexisting conditions that are exacerbated by pregnancy. One study of 255 patients with the condition found that 20% had had the lesions before they became pregnant.23 The tendency of the condition to be made markedly worse by pregnancy, however, leads us to include it here.