Recognizing, Diagnosing, and Managing Pregnancy Dermatoses.

Pregnancy dermatoses are inflammatory skin disorders that occur during pregnancy or immediately postpartum. This heterogenous group of disorders includes pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, atopic eruption of pregnancy, and pustular psoriasis of pregnancy. In this article, we provide a comprehensive literature review of each condition focusing on nomenclature, epidemiology, pathogenesis, clinical presentation, diagnosis, differential diagnosis, maternal risk, fetal risk, and treatment. We aim to increase awareness and help clinicians recognize, diagnose, and manage these unique conditions.

[Skin and pregnancy]. / Peau et grossesse.

Pregnancy has a substantial impact on the hormonal status of the organism, consequently influencing the physiology of the skin. This results in dermatoses that only occur during pregnancy, which can also improve or exacerbate pre-existing dermatoses. In this article, we explain the management of pregnancy-specific dermatoses : atopic eruption of pregnancy, polymorphic eruption of pregnancy, pemphigoid gestationis, impetigo herpetiformis, and intrahepatic cholestasis of pregnancy. It is essential to clearly distinguish these different dermatoses as some of them, such as pemphigoid gestationis, impetigo herpetiformis and intrahepatic cholestasis of pregnancy, can have fetal consequences and as result, need to be closely monitored by the obstetricians.

La grossesse a un impact considérable sur le statut hormonal de l'organisme, influençant ainsi la physiologie cutanée. Cela se traduit par des dermatoses qui ne se manifestent que pendant la grossesse. Cette dernière peut également améliorer ou exacerber des dermatoses préexistantes. Dans cet article, nous précisons la prise en charge des dermatoses spécifiques de la grossesse : l'eczéma atopique de la grossesse, l'éruption polymorphe gravidique, la pemphigoïde gestationnelle, l'impétigo herpétiforme et la cholestase intrahépatique gravidique. Il est important de distinguer ces dermatoses, puisque la pemphigoïde gestationnelle, l'impétigo herpétiforme et la cholestase intrahépatique gravidique présentent un risque fœtal et par conséquence nécessitent un suivi obstétrical rapproché.

Tratamiento con inmunoglobulinas intravenosas del penfigoide gestacional refractario al tratamiento convencional / Intravenous Immunoglobulin Therapy for Gestational Pemphigoid Refractory to Conventional Treatment

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Successful treatment of one case of pemphigoid gestationis complicating with polycystic ovary syndrome.

Pemphigoid gestationis (PG) is a rare, autoimmune, subepidermal bullous dermatosis associated with pregnancy. It is mainly caused by autoantibodies against hemidesmosomal proteins-bullous pemphigoid 180, and usually presents in the second or third trimester of pregnancy sometimes exacerbates spontaneously after delivery. Here we reported a case of PG with polycystic ovary syndrome (PCOS). This patient received assisted reproduction technology and suffered PG in her third trimester of pregnancy. To the best of our knowledge, there is no similar case has been reported.

Treatment Update of Autoimmune Blistering Diseases.

The treatment of refractory autoimmune blistering diseases (AIBDs) has always been a challenge. Because randomized controlled trials are lacking, treatment has been based on analysis of anecdotal data. The last 2 decades has seen the use of rituximab become a conventional treatment in the therapeutic armamentarium of AIBDs, leading to its Food and Drug Administration indication for pemphigus vulgaris in 2018. We review the current updated data on the use of rituximab including dosing, protocols, and its role in the algorithm of AIBDs. In addition, we discuss several promising novel emerging therapeutic agents for AIBDs.

[Specific dermatoses of pregnancy]. / Spezifische Schwangerschaftsdermatosen.

The specific dermatoses of pregnancy represent a heterogeneous group of inflammatory skin diseases related to pregnancy and/or the postpartum period. A clinically relevant classification has been well established over the past 10 years 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 a separate article (Kremer A, Ständer S, DOI 10.1007/s00105-016-3923-y ). Furthermore, we present a helpful algorithm for diagnosis and management of pruritus in pregnancy.

Itch in Pregnancy Management.

Pruritus is common in pregnancy. It deserves an elaborated work-up of the patient. It is frequently a symptom of a dermatosis that coincides by chance with pregnancy or a preexisting dermatosis that can flare during pregnancy. In some cases, it is due to the group of pregnancy-specific dermatoses. Work-up requires a prudent consideration of the diagnostic tests as well as the choice of treatment because of the potential effects on the fetus. This chapter will focus on the specific dermatoses of pregnancy and the local and systemic treatment of pruritus in general during pregnancy.

Dermatoses of pregnancy.

The dermatoses of pregnancy represent a distinct heterogenous group of cutaneous disorders that can impact the health of the pregnant woman and potentially the fetus. The current classification of pregnancy-specific cutaneous disorders is reviewed, along with important clinical features. Advances in management of these disorders, along with fetal implications, are discussed. The diagnosis of these disorders is challenging, but important clinical features can aid in diagnosis. There have been important advances in the management of these disorders and better understanding of potential fetal risks. Early recognition is critical for appropriate care.

[Dermatoses in pregnancy. Presentation of a case]. / Dermatosis del embarazo. A propósito de un caso.

Family physicians usually follow up pregnancies in which no special incidences are expected to occur. Cutaneous pruritus is a common symptom in pregnant women, on most occasions without further consequences. However, noteworthy is a group of very rare pathologies known as pregnancy dermatoses, some of which may have potentially severe complications, mainly for the fetus and the pregnancy outcome, and also, to a lesser degree, for the mothers and other future pregnancies. It is essential to know how to manage the pruritus, and how to take an adequate clinical history in order to diagnose these severe conditions. The case of a pregnant woman who consulted for pruriginous dermatoses (pemphigoid gestationis) is presented to illustrate this topic. A description of the diagnostic process, differential diagnosis, treatment and outcome, is included.

[Dermatoses of pregnancy]. / Terhességi dermatosisok.

Pregnancy is associated with hormonal, metabolic, immunologic, haemodynamic and vascular changes which produce physiological and pathological cutaneous manifestations. Most skin disorders during pregnancy represent physiological changes. The specific dermatoses of pregnancy are disease entities almost exclusively related to the pregnancy or the postpartum period. Some dermatoses characteristically improve or exacerbate during pregnancy. Dermatoses of pregnancy can potentially be a serious risk to the foetus. Although the foetal outcome is almost always favourable, early diagnosis and prompt treatment are essential. Use of any medication during pregnancy should be carefully considered, and as few medications as possible should be administered. In this review, the authors discuss the various dermatoses of pregnancy in detail, based on novel classification, aetiology and pathogenesis, diagnostic approach, disease course, prognosis, foetal risk and therapy.

Specific dermatoses of pregnancy and their treatment.

The specific dermatoses of pregnancy represent a diverse group of intensely pruritic dermatoses, occurring only in the puerperal state. The relative rarity of these conditions, the often variable clinical appearance, and the lack of definitive diagnostic tests have led to confusion regarding the appropriate diagnosis and management of the specific dermatoses of pregnancy. Herein we review the clinical characteristics, diagnosis and treatment of five dermatoses occurring during pregnancy: pruritic urticarial papules and plaques of pregnancy, atopic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis of pregnancy, and pustular psoriasis of pregnancy.

Glucocorticosteroid-resistant pemphigoid gestationis: successful treatment with adjuvant immunoadsorption.

A 40-year old prima para presented with multiple urticaria-like plaques and severe pruritus 2 weeks prior to giving birth by cesarean section. Three days after birth, the disease flared up and tense blisters appeared on hands, lower arms and feet. Based on the clinical presentation, direct immunofluorescence microscopy, complement binding test and detection of high levels of circulating anti-BP180 antibodies, the diagnosis of pemphigoid gestationis was established. Despite treatment with class IV topical corticosteroid and prednisolone p.o. up to 60 mg/day, both skin lesions and severe pruritus progressed accompanied by increasing anti-BP180 antibody serum levels. In order to continue breast feeding, the prednisolone dose could not be further increased and 10 immunoadsorptions over 4 weeks were performed. During this period, skin lesions cleared rapidly, pruritus subsided and BP180-specific serum autoantibodies decreased by 99.5% allowing the reduction of prednisolone to 7.5 mg/day. We conclude that immunoadsorption is an effective and safe adjuvant therapeutic option for severe pemphigoid gestationis.

Pemphigoid gestationis: a case report and review of the literature.

Pemphigoid gestationis (PG) is an uncommon autoimmune bullous disease that almost exclusively presents during pregnancy. Patients typically present with a diffuse blistering and intensely pruritic eruption that begins periumbilically and spreads to involve the rest of the body. Direct immunofluorescence demonstrating C3 in a linear pattern along the dermoepidermal junction confirms the diagnosis of PG. Corticosteroids remain the choice of therapy and early intervention is essential because of possible adverse effects of PG on the fetus. We report a case of PG and review the literature.

Pemphigoid gestationis: current management.

Pemphigoid gestationis is an autoimmune blistering disease of pregnancy caused by autoantibodies directed against BP180 (type XVII collagen) in the basement membrane zone. This article discusses the current management of this rare disease.