Dermatoses of Pregnancy - Clues to Diagnosis, Fetal Risk and Therapy

The specific dermatoses of pregnancy revisited and reclassified: Results of a retrospective two-center study on 505 pregnant patients

27 - Pregnancy Dermatoses

27 - Pregnancy Dermatoses

Pregnancy dermatoses describe a set of extremely pruritic dermatologic disorders that uniquely present in the gestational period. Those who care for pregnant women are left to try to differentiate these conditions. They are pruritic urticarial papules and plaques of pregnancy (PUPPP), pemphigoid gestationis, atopic eruption of pregnancy, and intrahepatic cholestasis of pregnancy. While the most common of these, atopic eruption of pregnancy and PUPPP, are benign, self-limiting diseases that have no implications for the fetus, pemphigoid gestationis and intrahepatic cholestasis of pregnancy both pose a risk to the fetus and are associated with adverse outcomes. A clinician’s ability to distinguish these conditions can impact both the mother and the fetus.

The specific dermatoses of pregnancy represent aheterogeneous group of inflammatory skin diseases related to pregnancy and/or the postpartum period. Aclinically relevant classification has been well established over the past 10years and includes pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. The hallmark of all four entities is severe pruritus that is accompanied by characteristic skin changes. While some of these dermatoses are distressing only to the mother because of pruritus, others may be associated with significant fetal risks. Early diagnosis and prompt treatment are therefore essential. In this review, we discuss in detail pemphigoid gestationis, polymorphic and atopic eruptions of pregnancy whereas intrahepatic cholestasis of pregnancy is discussed in aseparate article (Kremer A, Ständer S, DOI 10.1007/s00105-016-3923-y ). Furthermore, we present ahelpful algorithm for diagnosis and management of pruritus in pregnancy.

Introduction: Cutaneous findings in pregnancy can be physiologic, coincidental, alterations in pre-existing skin diseases or pregnancy specific. The Pregnancy dermatoses can impact the health of the pregnant woman and the fetus Objectives: Our objective was to determine the spectrum of skin disease associated with pregnancy and to identify the various types of pregnancy specific dermatoses and their fetal risk. Materials & methods: A prospective study was performed at dermatology out-patient department of Jumhoria hospital and Ibn Sina polyclinic, Benghazi-Libya over a period of 2 years. A total of 200 pregnant women presented with dermatological complaint were included. History, skin examination, and relevant investigations were performed. The patients with pregnancy specific dermatoses were followed up till delivery and the pregnancy outcome was recorded. The results were analyzed using SPSS. Results: Mean age was 32 years, 62% was multigravidae and 54% of pregnancy dermatoses occurred during third trimester. The highest number of cases presented with coincidental or preexisting diseases (71%); infections (34%), pre-existing skin diseases included eczema (13%), acne vulgaris (4%) and psoriasis (3%) Hyperpigmentation and strieagravidarum represented the main physiological changes (17%). Specific pregnancy dermatoses were present in 12%, these were intrahepatic cholestasis of pregnancy (4%), atopic eruption of pregnancy (3%), pemphigoid gestationis (3%) and polymorphic eruption of pregnancy (2%). Fetal complications including fetal mortality were reported with pemphigoid gestationis (30%) and intrahepatic cholestasis of pregnancy (25%). Conclusions: This study provides important data on the spectrum of pregnancy dermatoses in Libyan patients. Most of the reported pregnancy dermatoses were benign with no adverse effect on the fetus. Pemphigoid gestationis and intrahepatic cholestasis of pregnancy can be a source of significant fetal risk. To the best of our knowledge this is the first study carried out on pregnancy dermatoses in Benghazi, Libya.

Specific dermatoses of pregnancy are skin disorders that occur specifically during or immediately after pregnancy and cannot be found in non-pregnant patients. According to the current consensus, they include atopic eruption of pregnancy (AEP), polymorphic eruption of pregnancy (PEP), pemphigoid gestationis (PG), and intrahepatic cholestasis of pregnancy (ICP). The diagnosis of specific dermatoses of pregnancy can be challenging due to their variation in clinical presentation; moreover, the tests currently available do not always provide the clue for the diagnosis. However, some distinctive features may be helpful to differentiate between such entities. Accordingly, the knowledge of specific dermatoses of pregnancy and of their management is critical, since their early recognition may allow to provide care for the mother and prevent potential increased fetal risk. In fact, while AEP and PEP do not affect maternal and fetal prognosis, PG and, mainly, ICP are associated to maternal complications as well as the risk of fetal loss. In this paper, the epidemiology, pathogenesis, clinical features as well as management of AEP and PEP are reviewed in detail, while PG is described in another article of this issue. Moreover, the main features of ICP, which cannot be considered a primarily skin disease but may be managed first by dermatologists, are reported.

The current widely accepted classification for the dermatoses of pregnancy, based on the largest series to date, consists of four major categories: 1) polymorphic eruption of pregnancy (PEP), 2) atopic eruption of pregnancy (AEP), 3) pemphigoid gestationis (PG), and 4) intrahepatic cholestasis of pregnancy (ICP). Intrahepatic cholestasis of pregnancy, while not associated with any primary skin lesions, is currently accepted as one of the dermatoses of pregnancy. The most common skin disorder in pregnancy is atopic eruption of pregnancy. Pruritus represents a significant symptom in all four dermatoses. Differentiating among these entities, especially in their early stages, may pose a significant diagnostic challenge, requiring excluding each of the dermatoses methodically. Though not included in the current classification, impetigo herpetiformis (IH), a variant of pustular psoriasis, is frequently discussed together with dermatoses of pregnancy, considered by some as the fifth dermatosis of pregnancy. Striae gravidarum and melanoma in pregnancy will also be discussed in depth in this chapter. Multidisciplinary management involving a dermatologist expert in dermatologic conditions in pregnancy is of paramount importance.

The specific dermatoses of pregnancy represent heterogeneous group of pruritic skin disorders that occur exclusively in pregnancy. The updated classification proposed subdividing specific dermatoses of pregnancy into four main categories: 1. atopic eruption of pregnancy; 2. polymorphic eruption of pregnancy; 3. pemphigoid gestationis; 4. intrahepatic cholestasis of pregnancy. Severe pruritius, which is the main symptom in all 4 entities, can impair maternal quality of life. Significant maternal risks are not associated with specific dermatoses of pregnancy; however, pemphigoid gestationis and intrahepatic cholestasis of pregnancy are associated with fetal risks. Pathognomonic laboratory tests are not available, except direct immunofluorescence which is diagnostic of pemphigoid gestationis and elevated serum level of bile acids of intrahepatic cholestasis of pregnancy. Characteristic clinical features provide discrimination between polymorphic eruption of pregnancy and atopic eruption of pregnancy.

Introduction and purpose: Pregnancy is a remarkable and delicate period in a woman's life, marked by significant physiological adaptations to accommodate the growing fetus. These changes affect every organ system, including the skin. The aim of this review paper is to raise awareness and assist clinicians in recognizing, diagnosing, and effectively managing these distinct skin conditions associated with pregnancy. Material and methods: An extensive examination of articles published in scientific journals was carried out through online research platforms PubMed and Google Scholar. We searched articles by entering keywords in appropriate configuration: “pregnancy dermatoses”, “pemphigoid gestationis”, “ polymorphic eruption of pregnancy”, “intrahepatic cholestasis of pregnancy”, “pustular psoriasis of pregnancy”, “atopic eruption of pregnancy”. Description of the state of knowledge: This diverse set of pregnancy-related skin conditions includes pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, atopic eruption of pregnancy, and pustular psoriasis of pregnancy. Summary: Early diagnosis and treatment are crucial to mitigate maternal and fetal complications, and in some conditions, prevent fatalities. Improving clinicians' in-depth understanding of these disease processes can enhance patient safety and quality of life during and after pregnancy.

The specific dermatoses of pregnancy represent a recently reclassified heterogeneous group of pruritic inflammatory skin diseases unique to pregnancy that include pemphigoid gestationis, polymorphic eruption of pregnancy (PEP), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy (AEP). Among them, PEP and AEP are the most frequent ones. We performed a histopathological study of a series of PEP and AEP patients (n = 41). Twenty-two patients had PEP that started in the third trimester in 16 (73%) patients and postpartum in 6 (27%) patients. Histopathology revealed a superficial or superficial and deep perivascular dermatitis with eosinophils in all biopsies and signs of a lymphocytic vasculitis in 5 (23%) cases. Epidermal changes, including epidermal hyperplasia, spongiosis, and parakeratosis, occurred in 8 cases, in particular in elder lesions. Nineteen patients had AEP that started earlier [less than third trimester, 14 (74%) patients; third trimester, 5 (26%) patients]. Clinically, 5 (26%) patients showed eczematous lesions, 7 (37%) papular lesions, 3 (16%) presented both eczematous and prurigo lesions, and 4 (21%) experienced exacerbation of preexisting atopic dermatitis. Histopathologically, AEP was characterized by a perivascular lymphohistiocytic infiltrate with frequent eosinophils (74%) and epidermal changes in all but most of P-type biopsies. No definitive differential histopathological criteria between PEP and AEP were found. Only lymphocytic vasculitis with a mixed infiltrate with eosinophils was more frequent in PEP patients. Timing of onset, morphology of skin lesions, and a detailed clinicopathologic correlation are essential for diagnosis.

Pruritus in pregnancy – is it always obstetric cholestasis?

Dermatoses of pregnancy encompass a range of skin disorders that manifest uniquely during the gestational period, characterized by significant itching. This review delves into four primary types: polymorphic eruption of pregnancy, pemphigoid gestationis, atopic eruption of pregnancy, and intrahepatic cholestasis of pregnancy. While atopic eruption and polymorphic eruption are generally benign with no fetal impact, pemphigoid gestationis and intrahepatic cholestasis carry risks for the fetus, including prematurity and stillbirth. Key challenges include the difficulty in early diagnosis of pemphigoid gestationis due to its resemblance to polymorphic eruption and the increased risk of complications like gestational diabetes and pre-eclampsia in intrahepatic cholestasis. Understanding these dermatoses is crucial for prompt diagnosis and effective treatment, to prevent adverse outcomes for mothers and babies and improve the overall pregnancy experience. The review focuses on epidemiology, clinical features, diagnostic methods, maternal and fetal outcomes, treatment options, and comprehensive management strategies for these conditions.

During pregnancy, the mother undergoes changes to sustain and enable normal growth and development of the fetus. Common physiological changes include linea nigra, fibroepithelial polyps, striae, spider angioma, palmar erythema and pruritis gravidarum. However, there are some changes that are purely pathological, and these are termed the pregnancy-specific dermatoses (PSDs). The PSDs occur during pregnancy or in the immediate postpartum period. They do not include the various benign conditions or pre-existing dermatoses and tumours that may present or worsen with pregnancy. They do include a number of distinct and identifiable conditions: atopic eruption of pregnancy (AEP), polymorphic eruption of pregnancy (PEP), intrahepatic cholestasis of pregnancy (ICP) and pemphigoid gestationis (PG). These are a heterogeneous group of skin conditions characterized by pruritis and inflammatory changes. In addition, pruritis gravidarum is sometimes considered pathophysiological and thus part of this group, rather than a physiological process. Each of these conditions has a distinct, but not fully understood, pathogenesis. The mechanisms leading to PSD may be a reflection on the hormonal and immunological changes associated with pregnancy. AEP and PEP are benign conditions, and although they can cause distress to the mother, they are otherwise minor. However, ICP and PG are more serious conditions, and both carry the potential for serious risks to both the mother and the fetus. Thus, the pathophysiology of these latter two conditions is considered in more detail in the following article.

Pruritic Urticarial Papules and Plaques of Pregnancy