PRACTICE RECOMMENDATIONS
• Pemphigoid gestationis is best managed with oral prednisone at doses from 20 to 60 mg per day to control symptoms. B
• The pruritus associated with pruritic urticarial papules and plaques of pregnancy can be safely and effectively managed with topical corticosteroids and oral antihistamines. A
• Treat intrahepatic cholestasis of pregnancy with ursodeoxycholic acid, which likely reduces serum bile acids as well as associated fetal morbidity and mortality. B
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
The dermatoses of pregnancy are a poorly understood group of dermatologic conditions. The only thing they have in common is a tendency to appear during pregnancy.
Only 3 are considered unique to pregnancy, however; the others are probably exacerbations of preexisting conditions triggered by pregnancy. There isn’t even complete agreement on what to call them. And to make management even more complex, 2 patients—the mother and the fetus—need to be considered.
Who will manage these patients is another matter. These conditions fall into an overlapping area of health care, where family physicians, obstetricians, and dermatologists all have some share in the responsibility for diagnosis and treatment. As a family physician who probably cares for any number of pregnant patients on a weekly basis, you need to be sufficiently familiar with these conditions so that you can differentiate those that can be treated symptomatically and those that require a referral to a specialist. This review and handy TABLE will help you toward that end.
TABLE
Skin disorders of pregnancy: What you’ll see, how to treat
| Disorder | Lesions | Diagnosis and sequelae | Treatment | Recurrence |
|---|---|---|---|---|
| PG3,5 | Erythematous papules, progressing to vesicles, bullae; periumbilical distribution, sparing face, palms, and soles | Mean onset at 21 weeks; postpartum in 20% of cases. Direct immunofluorescence microscopy shows linear C3 deposition. Newborn may be small for gestational age, but no associated morbidity or mortality | Oral corticosteroids 20-60 mg/d, IVIG, or cyclosporine in refractory cases | Frequent. Skips a pregnancy 8% of the time |
| PUPPP8-10 | Urticarial papules and plaques on abdomen, legs, arms, buttocks, chest, and back | Usually present after 34th week, but can present at any stage. Diagnosis is clinical. No increase in fetal morbidity or mortality | Topical steroids and antihistamines | Uncommon |
| ICP14,17,19-22 | No primary lesions; secondary excoriations in any area patient can reach | Onset after 30th week in 80% of patients. Strongly indicated by serum bile acid >11 mcmol/L. Increased fetal mortality | Ursodeoxycholic acid 450-1200 mg/d | Frequent |
| EP/PP4,10,24 | Grouped, crusted erythematous papules, patches, and plaques, most commonly on extensor surfaces of arms and legs or on abdomen | Onset at any point in pregnancy. Clinical diagnosis. No increase in fetal morbidity or mortality | Symptomatic treatment with topical steroids or antihistamines | Frequent |
| APPP27-29 | Erythematous plaques and pustules starting on inner thighs and groin and spreading to trunk and extremities | Onset at any point in pregnancy. Clinical diagnosis by appearance of lesions and association with systemic illness. Increased incidence of miscarriage and stillbirth, and maternal mortality | Prednisone 15-60 mg/d, cyclosporine 100 mg twice daily in refractory cases, management of associated hypocalcemia | Unknown |
| PFP24,29 | Papules and pustules concentrated around hair follicles, often beginning on abdomen and spreading to extremities | Onset most often in third trimester. Clinical diagnosis. No associated fetal morbidity or mortality | Topical steroids | Unknown |
| APPP, acute pustular psoriasis of pregnancy; EP/PP, eczema of pregnancy/pruritus of pregnancy; ICP, intrahepatic cholestasis of pregnancy; IVIG, intravenous immunoglobulin; PFP, pruritic folliculitis of pregnancy; PG, pemphigoid gestationis; PUPPP, pruritic urticarial papules and plaques of pregnancy. | ||||
Dermatoses unique to pregnancy
Pemphigoid gestationis
Years ago, this disorder was referred to as herpes gestationis, because the lesions are herpetiform. Pemphigoid gestationis (PG) has an incidence of approximately 1 in 10,000 pregnancies.1,2 The time of onset is usually about the 21st week of gestation, although in about 20% of cases, the eruption appears immediately postpartum.3
Presentation. The disease usually begins with urticarial papules and plaques around the umbilicus and extremities. Bullous lesions tend to develop as the disease progresses, and are often not present on first presentation (FIGURE 1). PG lesions tend to spare the face, palms, and soles. Mucosal surfaces are involved in less than 20% of cases. In about 75% of cases, PG flares around the time of delivery, regressing spontaneously after the baby is born.4
Pathophysiology. The pathophysiology is nearly identical to that of bullous pemphigoid, a blistering skin disorder more often seen in elderly patients.5 Pemphigoid disorders are immune processes, involving an immunoglobulin G (IgG) immune response directed at a 180-kDa hemidesmosome transmembrane glycoprotein. This protein is the common target of several subepidermal blistering diseases.
Differential. Disorders that may have some of the same features as PG include pruritic urticarial papules and plaques of pregnancy (PUPPP), erythema multiforme, intrahepatic cholestasis of pregnancy (ICP), contact dermatitis, and drug reactions.