8.6 Pregnancy dermatoses

JA65.1

Disorders of Pregnancy. There are several synonyms depending on specific dermatoses.

Polymorphic eruption of pregnancy (PEP): Pruritic urticarial papules and plaques of pregnancy, pruritic eruption of pregnancy, toxemic rash of pregnancy.

Pemphigoid gestationis (PG): Gestational pemphigoid,  herpes gestationis.
 

Atopic eruption of pregnancy (AEP): Prurigo gestationis, papular dermatitis of pregnancy, pruritic folliculitis of pregnancy.

Intrahepatic cholestasis of pregnancy (ICP): Pruritus gravidarum, icterus gravidarum
 

Pustular psoriasis of pregnancy: Impetigo herpetiformis.

Dermatoses of pregnancy are a group of disorders seen throughout the world. They are usually seen more commonly in late pregnancy and in those twin or multiple pregnancies. Some of them may result in an increased fetal risk.

Polymorphic eruption of pregnancy (PEP): Represents the most common specific dermatosis of pregnancy. Incidence: 1:160 pregnancies.

Pemphigoid gestationis (PG): 1 in 40-50 000 pregnancies. Association with the haplotypes HLA-DR3 and HLADR4.

Pruritic folliculitis of pregnancy: incidence 1:3000 pregnancies.

Atopic eruption of pregnancy (AEP) is always causally linked with a personal and/or family atopic background.

Intrahepatic cholestasis of pregnancy (ICP): Geographic pattern. Positive family history  > 50% of cases.

Dermatoses that are specific to pregnancy, resulting from an interaction of multiple factors in the body during pregnancy. Include various types (see classification).

Polymorphic eruption of pregnancy (PEP): Pathogenesis unknown.

Pemphigoid gestationis: Autoimmune response against BP180 (bullous pemphigoid) antigen. Circulating complement-fixing IgG antibodies. Possible occurrence of the disease when using oral contraceptives or during the premenstrual period.

Atopic eruption of pregnancy:  May occur in atopic subjects or others without atopy.

Pruritic folliculitis of pregnancy: unknown.

Intrahepatic cholestasis of pregnancy:  Not a primary dermatosis but a pregnancy-related liver disorder with secondary cutaneous lesions in particular induced by scratching.

Pustular psoriasis of pregnancy: Impetigo herpetiformis is conconsidered a form of generalized pustular_psoriasis.

Depends on disorder. Skin eruptions usually appear in the third trimester of pregnancy and tend to resolve after delivery.

Polymorphic eruption of pregnancy (PEP): Benign, self-limited, pruritic, papulo-urticarial inflammatory disorder.  Clinically polymorphous intensely itchy erythematous papules and plaques within and/or adjacent to striae gravidarum,  macules, papulo-vesicules (eczematous), urticarial,  targetoid lesions similar to erythema multiforme; annular or polycyclic wheals; and rarely, small bullae excoriations and crusts.

• Typical onset: last trimester of pregnancy.
• Localization: Trunk and extremities.

Pemphigoid gestationis (PG): Intensely pruritic. Erythematous urticarial papules and plaques. The lesions progress to tense blisters and bullae.

• Typical onset: second or third trimester of pregnancy.
• Localization: Extremities and trunk, mostly on the abdomen (periumbilical).

Atopic eruption of pregnancy (AEP): Generalized pruritic eczematous skin condition in patients with an atopic diathesis.

• Typical onset: anytime during pregnancy.
• Localization: classic atopic predilection sites.

Prurigo of pregnancy: Excoriated papules and nodules

• Typical onset: middle of pregnancy.
• Localization: limbs and/or trunk.

Pruritic folliculitis of pregnancy: Follicular/ pustular and papular lesions.

• Typical onset: middle of pregnancy.
• Localization: limbs and/or trunk.

Intrahepatic cholestasis of pregnancy (ICP): Widespread pruritus. No primary skin lesions,  Secondary skin changes due to scratching.

• Typical onset: last trimester of pregnancy.
• Localization: widespread, limbs and/or trunk.

Pustular psoriasis of pregnancy (PPP)/ Impetigo herpetiformis: Tiny, superficial, pustules arranged in herpetiform distribution.

• Typical onset: last trimester of pregnancy.
• Localization: widespread, limbs and/or trunk; centrifugal spread.

See "Symptoms".

See "Symptoms".

Polymorphic eruption of pregnancy (PEP): Routine laboratory: normal.

Pemphigoid gestationis: Eosinophilia. HG factor. Immune serology (BP180 antigen).

Atopic eruption of pregnancy: Elevated serum IgE levels.

Intrahepatic cholestasis of pregnancy:  Elevation of the total serum bile acid levels.

Mild abnormalities of liver function tests: (elevated ALT, GGT, hyperbilirubinemia).

Pustular psoriasis of pregnancy:  Leukocytosis with neutrophilia, elevated erythrocyte sedimentation rate, hypoalbuminemia, and iron deficiency anemia.  Pustules are sterile.

Polymorphic eruption of pregnancy (PEP): Non-specific histologic features:  Spongiosis papillary dermal edema; superficial, mid-dermal perivascular lymphohistiocytic infiltrate with occasional eosinophils, DIF and IIF are negative.

Pemphigoid gestations:

• Pre-bullous stage: Perivascular inflammatory infiltrate, composed of lymphocytes, histiocytes, and eosinophils. Spongiosis and focal epidermal necrosis.
• Bullous stage: Subepidermal blister.

Direct immunofluorescence (IF): Linear deposition of C3 along the BMZ (basal membrane zone).

Indirect immunofluorescence/ELISA: Circulating IgG antibodies anti BP180.

Intrahepatic cholestasis of pregnancy: Skin biopsy: Non-specific changes. DIF and IIF: Negative.

Atopic eruption of pregnancy: Corresponding to atopic dermatitis; direct immunofluorescence: negative.

Pustular psoriasis of pregnancy: Subcorneal spongiform pustules, corresponding to to pustular psoriasis.

Most dermatoses of pregnancy resolve after delivery.  

Polymorphic eruption of pregnancy (PEP): The lesions usually resolve within 4–6 weeks.

Pemphigoid gestationis: Self-limiting. Spontaneous remission in weeks to months after delivery. Tends to reoccur in all subsequent pregnancies, usually with an earlier onset and increased severity.

Possible recurrences with subsequent pregnancies in atopic eruptions, intrahepatic cholestasis of pregnancy and pustular psoriasis of pregnancy.

Polymorphic eruption of pregnancy (PEP) and atopic eruption of pregnancy: The fetal and maternal prognoses are excellent in almost all cases.

Pemphigoid gestationis, intrahepatic cholestasis of pregnancy [ICP], and pustular psoriasis of pregnancy (PPP) are associated with considerable adverse fetal outcomes such as prematurity, fetal distress, or even stillbirth.

Intrahepatic cholestasis of pregnancy:  Increased risk of intra- and postpartum hemorrhage.

Pustular psoriasis of pregnancy:  Hypocalcemia, lymphadenopathy, seizures, and malaise, severe dehydration.

Clinical features, histopathological data and direct and indirect immunofluorescence studies. Immunopathologic tests (DIF/IIF) play an important role in differentiating pemphigoid gestationis from other bullous disorders. Onset of eruption.

In many instances should be established with different dermatoses appearing during pregnancy  and/or other related or specific dermatoses of pregnancy.

Polymorphic eruption of pregnancy (PEP): Pemphigoid gestationis (pre-bullous) or different dermatoses appearing during  pregnancy: Drug eruptions, allergic contact dermatitis, erythema multiforme, scabies.

Pemphigoid gestationis:

• Pre-blistering stage: Polymorphic eruption of pregnancy (PEP).
• Blistering stage:  Other autoimmune bullous diseases, bullous drug eruptions, or contact dermatitis coincident with pregnancy.

Atopic eruption of pregnancy: Pruritic dermatoses unrelated to and coinciding with gestation scabies, drug eruptions, and possible arthropod bites.

Intrahepatic cholestasis of pregnancy: Different dermatologic and/or internal conditions that cause pruritus in the absence of primary skin lesions.

Pustular psoriasis of pregnancy:  Autoimmune bullous dermatoses, acute generalized exanthematous pustulosis.

Polymorphic eruption of pregnancy (PEP):  Symptomatic treatment.

Pemphigoid gestationis: Potent topical corticosteroids. Systemic corticosteroids, to control blister formation, tapered and maintained at the lowest effective dose.  Immunosuppressive treatments only used after delivery.

Atopic eruption of pregnancy: Moderately potent topical corticosteroids and oral antihistamines.  Emollients or antipruritic additives such as menthol or polidocanol.

Intrahepatic cholestasis of pregnancy:  Antipruritic treatments: ursodeoxycholic acid, cholestyramine, antihistamines (non-sedative), anion exchange resins.

Pustular psoriasis of pregnancy:  TNF alpha blocker infusions, systemic corticosteroids, ciclosporin. After pregnancy, other agents used in psoriasis could be prescribed: PUVA, oral retinoids.  Antibacterials in case of secondary skin infection.

Fetal prognosis:

Pemphigoid gestationis, Intrahepatic cholestasis of pregnancy, pustular psoriasis of pregnancy: Increased risk to prematurity fetal distress (22–33%) and fetal abnormalities.