Skin Disease in Pregnancy: Identifying and Treating Common and Rare Presentations - Dermatology Advisor

Skin Disease in Pregnancy: Identifying and Treating Common and Rare Presentations

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  • Atopic eruption of pregnancy (AEP) is an umbrella term for atopic dermatitis (AD) of pregnancy, prurigo of pregnancy, and pruritic folliculitis of pregnancy.2,6 The 3 diseases are grouped together because of their similar appearance and significant overlap in histopathologic or laboratory findings. AEP is the most frequently diagnosed inflammatory skin disease in pregnant women.3 Three-quarters of cases manifest before the third trimester.3 Most women who develop AEP have little or no history of atopic skin changes prior to pregnancy. Patients typically present with eczematous patches or plaques on the face, trunk, or extensor surfaces of the limbs.3 Approximately one-third of patients develop papular lesions (prurigo or pruritic folliculitis), primarily on the trunk or limbs. Treatment generally consists of emollients, topical corticosteroids, and antihistamines, which are highly effective even in severe cases.2,5 The condition poses no risk to the fetus.3

    Atopic Eruption of Pregnancy

    Atopic eruption of pregnancy (AEP) is an umbrella term for atopic dermatitis (AD) of pregnancy, prurigo of pregnancy, and pruritic folliculitis of pregnancy.2,6 The 3 diseases are grouped together because of their similar appearance and significant overlap in histopathologic or laboratory findings. AEP is the most frequently diagnosed inflammatory skin disease in pregnant women.3 Three-quarters of cases manifest before the third trimester.3 Most women who develop AEP have little or no history of atopic skin changes prior to pregnancy.

    Patients typically present with eczematous patches or plaques on the face, trunk, or extensor surfaces of the limbs.3 Approximately one-third of patients develop papular lesions (prurigo or pruritic folliculitis), primarily on the trunk or limbs. Treatment generally consists of emollients, topical corticosteroids, and antihistamines, which are highly effective even in severe cases.2,5 The condition poses no risk to the fetus.3

  • Erythema nodosum (EN) is characterized by inflammation of subcutaneous fat cells.3 The condition is 3 to 6 times more common in women and typically occurs between age 25 and 40.14 Pregnancy is the cause of erythema nodosum (EN) in an estimated 2% to 6% of cases.2 Other causes include infection (especially streptococcal), sarcoidosis, and malignancy.14 During pregnancy, EN typically begins in the first trimester or early in the second trimester.2 Erythema nodosum is characterized by spontaneous eruption of firm red, tender, painful nodules on the lower legs (usually the shins) and occasionally on the forearms and thighs.3,14 The nodules typically reach 10 to 40 mm in diameter.12 Accompanying symptoms may include fever, ankle edema, and joint or abdominal pain.14 Most cases of EN resolve spontaneously within 4 to 8 weeks of onset.3,14 Compression stockings, bed rest with leg elevation, and analgesics can help with discomfort.2,3

    Erythema Nodosum

    Erythema nodosum (EN) is characterized by inflammation of subcutaneous fat cells.3 The condition is 3 to 6 times more common in women and typically occurs between age 25 and 40.14 Pregnancy is the cause of erythema nodosum (EN) in an estimated 2% to 6% of cases.2 Other causes include infection (especially streptococcal), sarcoidosis, and malignancy.14 During pregnancy, EN typically begins in the first trimester or early in the second trimester.2 Erythema nodosum is characterized by spontaneous eruption of firm red, tender, painful nodules on the lower legs (usually the shins) and occasionally on the forearms and thighs.3,14 The nodules typically reach 10 to 40 mm in diameter.12 Accompanying symptoms may include fever, ankle edema, and joint or abdominal pain.14 Most cases of EN resolve spontaneously within 4 to 8 weeks of onset.3,14 Compression stockings, bed rest with leg elevation, and analgesics can help with discomfort.2,3

  • Urticaria is common during pregnancy and presents as wheals, angioedema, or a combination of the two. Chronic cases last for 6 weeks or more.3,9 Urticaria is not specific to pregnancy, and data are insufficient to determine whether pregnancy improves or exacerbates the condition in women with preexisting chronic urticaria. However, chronic urticaria is twice as common in women, and evidence suggests sex hormones may affect urticaria in some women.2 Standard treatment in pregnant women with urticaria is oral antihistamines.2,9 No antihistamines have been shown to pose a risk for fetal harm. Chlorpheniramine, diphenhydramine, loratadine, and cetirizine are preferred.2,3 Expectant mothers should avoid using antihistamines late in pregnancy because they can stimulate uterine contractions and newborn infants may experience withdrawal.2

    Urticaria

    Urticaria is common during pregnancy and presents as wheals, angioedema, or a combination of the two. Chronic cases last for 6 weeks or more.3,9 Urticaria is not specific to pregnancy, and data are insufficient to determine whether pregnancy improves or exacerbates the condition in women with preexisting chronic urticaria. However, chronic urticaria is twice as common in women, and evidence suggests sex hormones may affect urticaria in some women.2 Standard treatment in pregnant women with urticaria is oral antihistamines.2,9 No antihistamines have been shown to pose a risk for fetal harm. Chlorpheniramine, diphenhydramine, loratadine, and cetirizine are preferred.2,3 Expectant mothers should avoid using antihistamines late in pregnancy because they can stimulate uterine contractions and newborn infants may experience withdrawal.2

  • Pityriasis rosea (PR) commonly occurs in males and females age 10 to 35.2 Although PR is not pregnancy-specific, it is more prevalent in pregnant women.2 Recent studies have concluded that PR is likely caused by reactivation of human herpes virus 6 or 7.2,11 Low levels of viral DNA have been found in the lesional skin, and experts believe the viruses do not directly infect skin cells but that PR is instead a reaction to viral replication.11 One study associated active human herpes virus 6 with preterm births and miscarriage.3 Pityriasis rosea first presents as an erythematous scaly plaque on the trunk or neck, sometimes called the herald patch. This is followed by an eruption of smaller plaques on the trunk and following the cleavage lines on the back in a sort of Christmas tree pattern.11 The rash typically clears within a few weeks to 2 months and does not require treatment.2,3 Pityriasis rosea is sometimes misdiagnosed as guttate psoriasis or tinea corporis.3

    Pityriasis Rosea

    Pityriasis rosea (PR) commonly occurs in males and females age 10 to 35.2 Although PR is not pregnancy-specific, it is more prevalent in pregnant women.2 Recent studies have concluded that PR is likely caused by reactivation of human herpes virus 6 or 7.2,11 Low levels of viral DNA have been found in the lesional skin, and experts believe the viruses do not directly infect skin cells but that PR is instead a reaction to viral replication.11 One study associated active human herpes virus 6 with preterm births and miscarriage.3

    Pityriasis rosea first presents as an erythematous scaly plaque on the trunk or neck, sometimes called the herald patch. This is followed by an eruption of smaller plaques on the trunk and following the cleavage lines on the back in a sort of Christmas tree pattern.11 The rash typically clears within a few weeks to 2 months and does not require treatment.2,3 Pityriasis rosea is sometimes misdiagnosed as guttate psoriasis or tinea corporis.3

  • Pustular psoriasis of pregnancy (PPP) is a rare, life-threatening disease.8 It is sometimes referred to as impetigo herpetiformis, but many dermatologists consider this a misnomer because PPP is not associated with the herpes virus or bacterial infection.8 Although it is unclear whether PPP is specific to pregnancy, it typically arises early in the third trimester in affected women.3,8 Sterile erythematous plaques studded by painful pustules originate in skin folds on the trunk and back before coalescing into large dry desquamating plaques that spread to the extremities.2,8 Symptoms include fever, nausea, and diarrhea. Patients may also have neutrophilia, electrolyte abnormalities, and elevated inflammatory markers.2,8 Aggressive treatment is required and usually consists of systemic corticosteroids.8 Untreated PPP can lead to placental insufficiency, intrauterine growth restriction, and even miscarriage or stillbirth.8 Pregnant women with PPP and their fetuses should be closely monitored.2,8

    Pustular Psoriasis of Pregnancy (Impetigo Herpetiformis)

    Pustular psoriasis of pregnancy (PPP) is a rare, life-threatening disease.8 It is sometimes referred to as impetigo herpetiformis, but many dermatologists consider this a misnomer because PPP is not associated with the herpes virus or bacterial infection.8 Although it is unclear whether PPP is specific to pregnancy, it typically arises early in the third trimester in affected women.3,8 Sterile erythematous plaques studded by painful pustules originate in skin folds on the trunk and back before coalescing into large dry desquamating plaques that spread to the extremities.2,8 Symptoms include fever, nausea, and diarrhea. Patients may also have neutrophilia, electrolyte abnormalities, and elevated inflammatory markers.2,8

    Aggressive treatment is required and usually consists of systemic corticosteroids.8 Untreated PPP can lead to placental insufficiency, intrauterine growth restriction, and even miscarriage or stillbirth.8 Pregnant women with PPP and their fetuses should be closely monitored.2,8

  • Pemphigoid gestationis (PG) is a rare pregnancy-related autoimmune disease that affects approximately 1 in 10,000 pregnant females.5 Typically, PG arises during the second trimester, becomes quiescent in the third trimester, and, in 75% of cases, flares up near delivery.2 PG is characterized by an intensely pruritic rash that typically arises in the umbilical region and is followed by papules and plaques.3 The rash spreads to almost the entire body surface, minus the face, palms, and soles.3 The lesions eventually progress to painful blisters.3 Definitive diagnosis requires a skin biopsy and immunofluorescence staining for tissue-bound immunoreactants.5 First-line treatment consists of oral corticosteroids, but intravenous immunoglobulin or cyclosporine may be used for refractory PG. More severe PG is associated with an increased risk for premature birth and small-for-gestational age babies.3 Autoantibodies can be transferred to the fetus, and 3% to 10% of newborn infants develop mild blisters that quickly resolve without treatment.3,5

    Pemphigoid Gestationis

    Pemphigoid gestationis (PG) is a rare pregnancy-related autoimmune disease that affects approximately 1 in 10,000 pregnant females.5 Typically, PG arises during the second trimester, becomes quiescent in the third trimester, and, in 75% of cases, flares up near delivery.2 PG is characterized by an intensely pruritic rash that typically arises in the umbilical region and is followed by papules and plaques.3 The rash spreads to almost the entire body surface, minus the face, palms, and soles.3 The lesions eventually progress to painful blisters.3 Definitive diagnosis requires a skin biopsy and immunofluorescence staining for tissue-bound immunoreactants.5 First-line treatment consists of oral corticosteroids, but intravenous immunoglobulin or cyclosporine may be used for refractory PG. More severe PG is associated with an increased risk for premature birth and small-for-gestational age babies.3 Autoantibodies can be transferred to the fetus, and 3% to 10% of newborn infants develop mild blisters that quickly resolve without treatment.3,5

  • Sweet syndrome affects males and females of all ages, but most cases occur in individuals age 30 to 50.13 Sweet syndrome is 3 times more common in females. It is considered idiopathic, but possible triggers include infection, malignancy, autoimmune disorders, and certain medications.13 Approximately 2% of cases are related to pregnancy; it most often arises during the first or second trimester.2 At onset, patients have sudden eruptions of tender, painful plaques or plaque-like nodules.13 The lesions usually appear on the face, neck, trunk, and limbs but occasionally affect the mouth, genitals, and eyes. Common symptoms include fever and arthralgia. Histopathologic examination will reveal neutrophilic infiltrates. Laboratory findings often include leukocytosis, neutrophilia, and elevated erythrocyte sedimentation rate.13 Most cases resolve fairly quickly without treatment, but topical or oral corticosteroids can be used. Sweet syndrome does not appear to harm fetal health.2

    Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis)

    Sweet syndrome affects males and females of all ages, but most cases occur in individuals age 30 to 50.13 Sweet syndrome is 3 times more common in females. It is considered idiopathic, but possible triggers include infection, malignancy, autoimmune disorders, and certain medications.13 Approximately 2% of cases are related to pregnancy; it most often arises during the first or second trimester.2

    At onset, patients have sudden eruptions of tender, painful plaques or plaque-like nodules.13 The lesions usually appear on the face, neck, trunk, and limbs but occasionally affect the mouth, genitals, and eyes. Common symptoms include fever and arthralgia. Histopathologic examination will reveal neutrophilic infiltrates. Laboratory findings often include leukocytosis, neutrophilia, and elevated erythrocyte sedimentation rate.13 Most cases resolve fairly quickly without treatment, but topical or oral corticosteroids can be used. Sweet syndrome does not appear to harm fetal health.2

  • Sarcoidosis is a granulomatous disorder that can affect many organs, including the skin. Sarcoidosis is not caused by and is not unique to pregnancy. However, in women with sarcoidosis, onset typically occurs between age 30 and 40.2 Sarcoidosis affects an estimated 1 in 1500 to 2000 pregnancies and is more common in black women.2,12 It is T<SUBH1-mediated, and case reports suggest pregnancy-induced elevation in estrogen levels is beneficial for patients.12 Management of sarcoidosis depends on which organs are involved. For cutaneous sarcoidosis, topical corticosteroids are usually sufficient but systemic corticosteroids may be required for acute flares.2 Hydroxychloroquine has been used safely in pregnant women with lupus and may be an effective approach in systemic sarcoidosis.2 Reports about the effect of sarcoidosis on fetal health are conflicted. Some data suggest women with sarcoidosis have an increased risk for preeclampsia, eclampsia, thrombotic events, and premature delivery.2

    Sarcoidosis

    Sarcoidosis is a granulomatous disorder that can affect many organs, including the skin. Sarcoidosis is not caused by and is not unique to pregnancy. However, in women with sarcoidosis, onset typically occurs between age 30 and 40.2 Sarcoidosis affects an estimated 1 in 1500 to 2000 pregnancies and is more common in black women.2,12 It is T<SUBH1-mediated, and case reports suggest pregnancy-induced elevation in estrogen levels is beneficial for patients.12

    Management of sarcoidosis depends on which organs are involved. For cutaneous sarcoidosis, topical corticosteroids are usually sufficient but systemic corticosteroids may be required for acute flares.2 Hydroxychloroquine has been used safely in pregnant women with lupus and may be an effective approach in systemic sarcoidosis.2 Reports about the effect of sarcoidosis on fetal health are conflicted. Some data suggest women with sarcoidosis have an increased risk for preeclampsia, eclampsia, thrombotic events, and premature delivery.2

  • Psoriasis is a chronic disease that affects 2% to 3% of men and women.2 The disease is characterized by plaques and reddish scaly patches. Some women develop pustular psoriasis, which can be life-threatening if severe.3 Although most women with psoriasis notice symptom improvement during pregnancy, 10% to 20% have symptom deterioration.3 The effect of psoriasis on fetal health is unclear.2 Some studies have suggested that psoriasis, which is a TH1-driven disease, contributes to lower birth weight as observed in other TH1-driven diseases.2 The proposed negative effects of psoriasis on fetal health are usually linked to moderate or severe psoriasis. Topical corticosteroids are safe to use during pregnancy, but topical tar products, tazarotene, and retinoids are teratogenic and should be avoided.2,7 Ultraviolent B phototherapy is safe in pregnant women with moderate to severe psoriasis but may compromise folate levels.2 Limited data are available about the safety of biologics during pregnancy, and careful consideration is required before prescribing.2,7

    Psoriasis Vulgaris

    Psoriasis is a chronic disease that affects 2% to 3% of men and women.2 The disease is characterized by plaques and reddish scaly patches. Some women develop pustular psoriasis, which can be life-threatening if severe.3 Although most women with psoriasis notice symptom improvement during pregnancy, 10% to 20% have symptom deterioration.3 The effect of psoriasis on fetal health is unclear.2 Some studies have suggested that psoriasis, which is a TH1-driven disease, contributes to lower birth weight as observed in other TH1-driven diseases.2 The proposed negative effects of psoriasis on fetal health are usually linked to moderate or severe psoriasis. Topical corticosteroids are safe to use during pregnancy, but topical tar products, tazarotene, and retinoids are teratogenic and should be avoided.2,7 Ultraviolent B phototherapy is safe in pregnant women with moderate to severe psoriasis but may compromise folate levels.2 Limited data are available about the safety of biologics during pregnancy, and careful consideration is required before prescribing.2,7

  • The causes of erythema annulare centrifugum (EAC) are unclear. Experts have suggested EAC is a hypersensitive reaction to any number of potential triggers, such as pathogens (eg, viruses, bacteria, fungi, or parasites), malignancy, autoimmune disease, certain foods, and various medications. Pregnancy is a rare trigger of EAC.2,10 Often, no cause is found and EAC is classified as idiopathic.10 EAC begins as annular or polycyclic lesions, which typically arise on the trunk and extremities.10 The lesions grow 2 to 3 mm per day and generally stop at approximately 10 cm.10 The lesions typically contain papules, which begin to clear once the lesion stops growing, and a fine, trailing scale inside the lesion’s border develops.10 Lesions may be pruritic in some patients. Topical corticosteroids and petrolatum ointment have been used to treat pregnant women with idiopathic EAC.2 In cases in which the cause is known, EAC should resolve with treatment of the precipitating condition.10

    Erythema Annulare Centrifugum

    The causes of erythema annulare centrifugum (EAC) are unclear. Experts have suggested EAC is a hypersensitive reaction to any number of potential triggers, such as pathogens (eg, viruses, bacteria, fungi, or parasites), malignancy, autoimmune disease, certain foods, and various medications. Pregnancy is a rare trigger of EAC.2,10 Often, no cause is found and EAC is classified as idiopathic.10

    EAC begins as annular or polycyclic lesions, which typically arise on the trunk and extremities.10 The lesions grow 2 to 3 mm per day and generally stop at approximately 10 cm.10 The lesions typically contain papules, which begin to clear once the lesion stops growing, and a fine, trailing scale inside the lesion’s border develops.10 Lesions may be pruritic in some patients. Topical corticosteroids and petrolatum ointment have been used to treat pregnant women with idiopathic EAC.2 In cases in which the cause is known, EAC should resolve with treatment of the precipitating condition.10

Many dermatologic changes occur during pregnancy. Most are due to typical physiological or hormonal changes that occur during gestation and are benign. 1 These primarily cosmetic changes affect pigmentation or vascular appearance and do not require treatment. However, many women acquire pregnancy-specific inflammatory skin conditions (dermatitises) or experience worsening or improvement of preexisting dermatitises during gestation. 1 The effect of pregnancy on inflammatory skin disorders is due largely to profound changes in the maternal immune system, with a transition in the balance of T-helper lymphocytes from predominantly T-helper 1 (TH1) to T-helper 2 (T H2).2 The resulting changes in cytokine production protect the fetus from rejection but also increase the woman’s risk for skin disease. 3

It is important to examine pregnant patients with pruritis for any skin conditions that might be caused or exacerbated by the pregnancy.4 Dermatitises can behave unpredictably during gestation and must be managed carefully to ensure the well-being of mother and fetus. 2,5

Compiled by Garret Melton

References

  1. Sävervall C, Sand FL, Thomsen SF. Dermatological diseases associated with pregnancy: pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. Dermatol Res Pract. 2015;2015:979635.
  2. Yang CS, Teeple M, Muglia J, Robinson-Bostom L. Inflammatory and glandular skin disease in pregnancy. Clin Dermatol. 2016;34(3):335-343.
  3. Jones SV, Ambros-Rudolph C, Nelson-Piercy C. Skin disease in pregnancy. BMJ. 2014;348:g3489.
  4. Kannambal K, Tharini GK. A screening study on dermatoses in pregnancy. J Clin Diagn Res. 2017;11(5):WC01-WC05.
  5. Bremmer M, Driscoll MS, Colgan R. 6 skin disorders of pregnancy: a management guide. OBG Management. 2010;22(6):24-33.
  6. Kurien G, Badri T. Dermatoses of pregnancy. Treasure Island, Florida: StatPearls Publishing; 2018.
  7. Hoffman MB, Farhangian M, Feldman SR. Psoriasis during pregnancy: characteristics and important management and recommendations. Expert Rev Clin Immunol. 2015;11(6):709-720.
  8. Trivedi MK, Vaughn AR, Murase JE. Pustular psoriasis of pregnancy: current perspectives. Int J Womens Health. 2018;10:109-115.
  9. Ensina LF, Cusato-Ensina AP, Camelo-Nunes IC, Solé D. Omalizumab as third-line therapy for urticaria during pregnancy. J Investig Allergol Clin Immunol. 2017;27(5):326-327.
  10. McDaniel B, Cook C. Erythema annulare centrifigum. Treasure Island, Florida: StatPearls Publishing; 2018.
  11. Mahajan K, Relhan V, Relhan AK, Garg VK. Pityriasis rosea: an update on etiopathogenesis and management of difficult aspects. Indian J Dermatol. 2016;61(4):375-384.
  12. Cozier YC, Berman JS, Palmer JR, et al. reproductive and hormonal factors in relation to incidence of sarcoidosis in us black women. Am JEpidemiol. 2012;176(7):635-641.
  13. Priyanka V, Hearth Holmes MP. Sweet syndrome. Treasure Island, Florida: StatPearls Publishing; 2017.
  14. Hafsi W, Koya HH. Erythema, nodosum. Treasure Island, Florida: StatPearls Publishing; 2017.
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