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      Autoimmune blistering diseases in females: a review ☆☆

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          Abstract

          The autoimmune blistering diseases (AIBDs) are a group of heterogeneous skin diseases with autoantibodies directed against structural proteins in the skin. A new interest in the female bias towards autoimmune diseases in general has led to our attention to focus on how and why this female bias manifests in AIBD. The authors aim to review and explore the various aspects of AIBD affecting females more than males, including the higher prevalence, worse quality of life, and complex management issues such as pregnancy and lactation.

          Capsule summary

          What is already known on this topic?

          • Echoing autoimmune diseases in general, most autoimmune blistering diseases (AIBDs) have a female predominance, but the exact level of predominance is unknown.

          • Pregnancy raises several complicated management issues for females with an AIBD.

          What does this article add to our knowledge?

          • Review of sex-specific epidemiology and etiology of each AIBD.

          • Exploration and explanation of the key factors underlying the detrimental impacts of AIBD on women’s quality of life (QOL).

          • Discussion of management issues in pregnancy and lactation for females with an AIBD.

          How does this information impact clinical practice and/or change patient care?

          • An awareness and understanding of the female predominance in AIBDs will ensure more appropriate diagnosis, evaluation, and future research.

          • Emphasizing holistic care targeting the debilitating effects of AIBDs on women’s QOL.

          • Informing the reader of optimal, yet safe interventions for pregnant women with an AIBD.

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          Most cited references113

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          Sex differences in autoimmune disease.

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            SeXX matters in immunity.

            The significant contributions of sex to an immune response, specifically in the context of the sex bias observed in susceptibility to infectious and autoimmune diseases and their pathogenesis, have until recently, largely been ignored and understudied. This review highlights recent findings related to sex-specific factors that provide new insights into how sex determines the transcriptome, the microbiome, and the consequent immune cell functional profile to define an immune response. Unquestionably, accumulating data confirm that sex matters and must be a consideration when decisions around therapeutic intervention strategies are developed. Copyright © 2013 Elsevier Ltd. All rights reserved.
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              Birth defects after maternal exposure to corticosteroids: prospective cohort study and meta-analysis of epidemiological studies.

              Corticosteroids are first-line drugs for the treatment of a variety of conditions in women of childbearing age. Information regarding human pregnancy outcome with corticosteroids is limited. We collected prospectively and followed up 184 women exposed to prednisone in pregnancy and 188 pregnant women who were counseled by Motherisk for nonteratogenic exposure. The primary outcome was the rate of major birth defects. A meta-analysis of all epidemiological studies was conducted. The Mantel-Haenszel summary odds ratio was calculated for the pooled studies with 95% confidence intervals. A cumulative summary odds ratio was also calculated by combining studies in chronological order. Chi-squared for homogeneity was determined to establish the comparability of the studies. In our prospective study, there was no statistical difference in the rate of major anomalies between the corticosteroid-exposed and control groups. In the meta-analysis, the Mantel-Haenszel summary odds ratio for major malformations with all cohort studies was 1.45 [95% CI 0.80, 2.60] and 3.03 [95% CI 1.08, 8. 54] when Heinonen et al. ('77) was removed. This suggests a marginally increased risk of major malformations after first-trimester exposure to corticosteroids. In addition, summary odds ratio for case-control studies examining oral clefts was significant (3.35 [95% CI 1.97, 5.69]). Although prednisone does not represent a major teratogenic risk in humans at therapeutic doses, it does increase by an order of 3.4-fold the risk of oral cleft, which is consistent with the existing animal studies. Copyright 2000 Wiley-Liss, Inc.
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                Author and article information

                Contributors
                Journal
                Int J Womens Dermatol
                Int J Womens Dermatol
                International Journal of Women's Dermatology
                Elsevier
                2352-6475
                26 February 2015
                February 2015
                26 February 2015
                : 1
                : 1
                : 4-12
                Affiliations
                Department of Dermatology St George Hospital, Sydney, Australia
                University of New South Wales Sydney, Australia
                Author notes
                [* ]Corresponding author. d.murrell@ 123456unsw.edu.au
                Article
                S2352-6475(15)00012-X
                10.1016/j.ijwd.2015.01.002
                5418673
                28491949
                2c0aa424-c2b3-4c70-89eb-b4fe562407b3
                © 2015 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 13 September 2014
                : 23 October 2014
                : 13 January 2015
                Categories
                Original Research

                autoimmune blistering diseases,epidemiology,epidermolysis bullosa acquisita,female,pemphigoid,pemphigus,quality of life,toxic epidermal necrolysis,treatment

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