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    Pemphigoid gestationis successfully treated with intravenous immunoglobulin.
    BMJ Case Rep 2018 Apr 7;2018. Epub 2018 Apr 7.
    Department of Dermatovenereology, Hospital de Braga, Braga, Portugal.
    Pemphigoid gestationis (PG), also known as , is a rare autoimmune blistering disease specific to pregnancy, which usually presents in the second or third trimesters and, in 15%-25% of cases, during the immediate postpartum period.Although the ethiopathogeny of PG is not fully clarified, most patients develop antibodies against a 180 kDa transmembrane hemidesmosomal protein (BP180; BPAG2; collagen XVII). PG has a strong association with human leucocyte antigens DR3 and DR4. Read More

    Pemphigoid gestationis: a case series and review of the literature.
    J Dermatolog Treat 2018 Apr 5:1-17. Epub 2018 Apr 5.
    a Department of Dermatology , Wake Forest School of Medicine , Winston-Salem , NC , USA.
    Pemphigoid gestationis (PG) is a rare autoimmune disease associated with pregnancy. Clinically, it presents with severe pruritus that precedes the appearance of vesiculobullous lesions. It usually begins on the trunk and spread rapidly to other parts of the body. Read More

    A subset of patients with pemphigoid (herpes) gestationis has serological evidence of celiac disease.
    Int J Dermatol 2018 May 23;57(5):534-540. Epub 2018 Feb 23.
    Immunodermatology Laboratory, Department of Dermatology, School of Medicine, University of Utah, Salt Lake City, UT, USA.
    Background: Pemphigoid (herpes) gestationis (PG) is an uncommon, self-limited disease with other autoimmune associations; however, celiac disease (CD) is not recognized as one.

    Methods: From 71 patients' sera submitted for herpes gestationis factor (HGF) testing over a 5-year period, 12 were consistent with PG demonstrating HGF and increased IgG BP180 antibody levels; these sera were tested for IgA and IgG endomysial antibodies (EMA), epithelial basement membrane zone and cell surface antibodies by indirect immunofluorescence, and for IgA and IgG tissue transglutaminase (transglutaminase 2 or TG2) antibodies, IgA epidermal transglutaminase (transglutaminase 3 or TG3) antibodies, IgG BP230, and IgG desmoglein 1 and desmoglein 3 antibodies by enzyme-linked immunosorbent assays (ELISAs).

    Results: Three of 12 patients' sera with PG (25%) had CD antibodies with positive IgA EMA and increased IgA TG2 antibody levels; two of these had positive IgG EMA, and one other had an increased IgA TG3 antibody level. Read More

    Treatment of Autoimmune Bullous Disorders in Pregnancy.
    Am J Clin Dermatol 2018 Feb 2. Epub 2018 Feb 2.
    Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA.
    Autoimmune bullous diseases (AIBD), including pemphigus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, and pemphigoid gestationis, pose significant therapeutic challenges, especially in pregnant and post-partum breastfeeding patients or those planning to conceive. Data on the safety and efficacy of therapeutic interventions during the perinatal period are lacking because randomized controlled trials are typically not performed in this setting. However, many of the treatments for AIBD are also used in other diseases, so data can be extrapolated from studies or case reports in these other patient populations. Read More

    Spectrum of Autoimmune Bullous Diseases in Northern Greece. A 4-year Retrospective Study and Review of the Literature.
    Acta Dermatovenerol Croat 2017 Oct;25(3):195-201
    Foteini Lamprou, MD, MSc, PhD candidate, 2nd University Department of Dermatology Papageorgiou General Hospital Aristotle University of Thessaloniki, Ring Road, Municipality of Pavlos Melas, Area of N. Efkarpia, 56403 Thessaloniki, Greece;
    Bullous Diseases Unit at the 2nd Department of Dermatology and Venereology, Aristotle University of Thessaloniki was founded with the aim to provide the optimal diagnostic approach and treatment of patients with autoimmune bullous diseases (AΙBD). We processed all AIBD files of patients diagnosed from 2011 to 2014 in order to record all epidemiological data and therapeutic manipulations during monitoring. 57 patients were diagnosed with intraepidermal and 62 with subepidermal bullous diseases. Read More

    Case of epidermolysis bullosa acquisita with concomitant anti-laminin-332 antibodies.
    J Dermatol 2018 Apr 4;45(4):472-474. Epub 2017 Dec 4.
    Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
    Subepidermal autoimmune blistering disease including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, anti-laminin-γ1 pemphigoid, linear immunoglobulin A bullous disease and epidermolysis bullosa acquisita (EBA), are all characterized by direct immunofluorescence microscopy or immunoglobulin deposition on the basement membrane zone. Among them, EBA is a rare acquired subepidermal autoimmune blistering disease of the skin and mucous membranes reactive with type VII collagen, a major component of the epidermal basement membrane zone. Anti-laminin-332-type mucous membrane pemphigoid has pathogenic autoantibodies against laminin-332, which is a basement membrane heterotrimeric protein composed of α3, β3 and γ2 laminin chains. Read More

    Pemphigoid gestationis: current perspectives.
    Clin Cosmet Investig Dermatol 2017 8;10:441-449. Epub 2017 Nov 8.
    Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark.
    Many skin diseases can occur in pregnant women. However, a few pruritic dermatological conditions are unique to pregnancy, including pemphigoid gestationis (PG). As PG is associated with severe morbidity for pregnant women and carries fetal risks, it is important for the clinician to quickly recognize this disease and refer it for dermatological evaluation and treatment. Read More

    Subepidermal autoimmune bullous diseases: overview, epidemiology, and associations.
    Immunol Res 2018 Feb;66(1):6-17
    Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel.
    Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different regions of the world. Read More

    The Value of Direct Immunofluorescence on Proteinase-Digested Formalin-Fixed Paraffin-Embedded Skin Biopsies.
    Am J Dermatopathol 2018 Feb;40(2):111-117
    Department of Pathology, University of Massachusetts Medical School, UMass Memorial Medical Center, Worcester, MA.
    Direct immunofluorescence (DIF) on frozen tissue (DIF-F) is the method of choice for the identification of immune deposits present in skin and other tissues. DIF can also be performed on formalin-fixed paraffin-embedded tissue (DIF-P) after antigen retrieval with proteases and has proven to be of value in renal pathology. However, its utility in skin biopsies has not been fully examined. Read More

    Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.
    Clin Rev Allergy Immunol 2018 Feb;54(1):26-51
    Department of Dermatology, University of Bern, Bern, Switzerland.
    Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. Read More

    Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder.
    Australas J Dermatol 2017 May 19. Epub 2017 May 19.
    Department of Neurology, Mohammed V. Rabat Military Hospital, Rabat, Morocco.
    Pemphigoid gestationis is a skin-specific autoimmune disorder that can sometimes present as the cutaneous manifestation of a multiorgan disease due to potentially common pathogenic mechanisms. We report a severe form of pemphigoid gestationis in a 32-year-old primigravida woman, who presented at 22 weeks of gestation with headaches and blurred vision, later developing encephalitis, intrauterine fetal demise and dilated cardiomyopathy. Read More

    A resistant case of pemphigus gestationis successfully treated with cyclosporine.
    Interv Med Appl Sci 2016 Mar;8(1):20-22
    Department of Obstetrics and Gynecology, Ankara Numune Education and Research Hospital , Ankara , Turkey.
    Pemphigoid gestationis (PG) is a rare autoimmune blistering disease of pregnancy caused by antibasement membrane zone auto-antibodies. The usual clinical findings are multiple pruritic urticarial papules and plaques, target lesions, vesicles, and blisters that occur during the second and third trimesters of pregnancy or in the immediate postpartum period. The disease is often treated with topical corticosteroids and oral antihistaminics. Read More

    Vesicular Contact Reaction May Progress into Erythema Multiforme.
    Acta Dermatovenerol Croat 2016 Dec;24(4):307-309
    Prof. Magdalena Czarnecka-Operacz, MD, PhD, Department of Dermatology, 49 Przybyszewski Str., 60-355 Poznań, Poland.
    Dear Editor, Erythema multiforme is considered an acute skin condition, characterized by a self-limiting and sometimes recurrent course. It is regarded as a type IV hypersensitivity reaction associated with certain infections, medications, and other various triggers. Allergic contact dermatitis is in turn a delayed type of induced allergy as a result of cutaneous contact with a specific allergen to which the patient develops specific sensitivity. Read More

    Bullous, pseudobullous, & pustular dermatoses.
    Semin Diagn Pathol 2017 May 14;34(3):250-260. Epub 2016 Dec 14.
    Section of Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, United States. Electronic address:
    Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Read More

    [Specific dermatoses of pregnancy].
    Hautarzt 2017 Feb;68(2):87-94
    Hautklinik, Universitätsklinikum Erlangen, Ulmenweg 18, 91054, Erlangen, Deutschland.
    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. Read More

    Polymorphic eruption of pregnancy: Review of literature.
    J Obstet Gynaecol 2017 Feb 14;37(2):137-140. Epub 2016 Dec 14.
    a Department of Obstetrics and Gynaecology , Centro Hospitalar Tâmega e Sousa, Hospital Padre Américo , Penafiel , Portugal.
    Polymorphic Eruption of Pregnancy (PEP) is one of the most common dermatosis related to pregnancy. PEP usually consists of pruritic papules and plaques appearing in the third trimester of pregnancy. It is more common in primigravidae and twin pregnancies. Read More

    Successful treatment of a severe early onset case of pemphigoid gestationis with intravenous immunoglobulin in a twin pregnancy conceived with in vitro fertilisation in a primigravida.
    J Obstet Gynaecol 2017 Feb 6;37(2):246-247. Epub 2016 Dec 6.
    a Department of Obstetrics and Gynaecology. Maternal-Fetal Medicine Unit , Universitary La Paz Hospital , Madrid , Spain.

    Pregnancy with Pemphigoid Gestationis: A Rare Entity.
    J Clin Diagn Res 2016 Jul 1;10(7):QD06-7. Epub 2016 Jul 1.
    Senior Specialist, Department of Obstetrics and Gynecology, Babu Jagjivan Ram Hospital , Delhi, India .
    Pemphigoid Gestationis (PG) is a rare autoimmune blistering disease with an incidence of 1/50000 pregnancies. Presentation in the second or third trimester is most common and tends to recur in subsequent pregnancies with earlier onset and a more severe course. Direct Immunofluorescence (DIF) staining is confirmatory on skin biopsy specimen. Read More

    Bullous impetigo and pregnancy: Case report and review of blistering conditions in pregnancy.
    Dermatol Online J 2016 Apr 18;22(4). Epub 2016 Apr 18.
    Department of Dermatology, University of California San Diego.
    Background:  Bullous impetigo results from Staphylococcus aureus (S. aureus) release of exfoliative toxins type A and type B thatresults in flaccid, easily ruptured, bullae in the upper layers of the epidermis.  Physiologic, gestation-associated, and incidental skin changes can occur in pregnancy. Read More

    Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis.
    Cutis 2016 Jun;97(6):E20-4
    Department of Dermatology, University of Puerto Rico School of Medicine, Río Piedras.
    Lichen planus pemphigoides (LPP) is a rare condition characterized by tense blisters that arise on lesions of lichen planus (LP) and on unaffected skin. We present the case of a 25-year-old pregnant woman at 12 weeks' gestation who developed an acute bullous eruption after 5 months of worsening LP. Similarities to pemphigoid gestationis (PG) included lesions around the periumbilical area and multiple urticarial erythematous papules and plaques in addition to linear C3 and IgM deposition along the basement membrane zone (BMZ) on direct immunofluorescence (DIF). Read More

    Polymorphic eruption of pregnancy.
    Clin Dermatol 2016 May-Jun;34(3):383-91. Epub 2016 Feb 9.
    Department of Dermatology, University of Illinois at Chicago College of Medicine, Chicago, Illinois.
    Polymorphic eruption of pregnancy (PEP), a specific dermatosis of pregnancy also known as pruritic urticarial papules and plaques of pregnancy (PUPPP), is a benign, self-limited skin disorder. Key features include an increased prevalence in primigravidas, onset in the third trimester, remission near the time of delivery, and association with multiple gestation pregnancy. The clinical features are crucial to diagnosis. Read More

    Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis.
    Clin Dermatol 2016 May-Jun;34(3):378-82. Epub 2016 Feb 9.
    Department of Dermatology, Allergy, and Venereology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.
    Pemphigoid gestationis (PG) is the only autoimmune disease exclusively emerging in pregnancy. It belongs to the pemphigoid group of disorders, a class of autoimmune blistering skin diseases featuring an immune response against different hemidesmosomal proteins. PG is caused by a break of immunotolerance against the hemidesmosomal protein BP180. Read More

    Skin disease in pregnancy: The approach of the obstetric medicine physician.
    Clin Dermatol 2016 May-Jun;34(3):320-6. Epub 2016 Feb 10.
    Department of Dermatology, Warren Alpert Medical School of Brown University, Providence, Rhode Island. Electronic address:
    This review presents the approach of the obstetric medicine physician to skin disease in pregnancy. It elaborates on common skin-related problems during gestation, such as pruritus, with or without eruption, and drug eruptions. An algorithmic approach to the differential diagnosis of pruritus in pregnancy is outlined. Read More

    Dermatoses of pregnancy: Nomenclature, misnomers, and myths.
    Clin Dermatol 2016 May-Jun;34(3):314-9. Epub 2016 Feb 9.
    Department of Dermatology, University of California School of Medicine, San Francisco, California; Department of Dermatology, Palo Alto Foundation Medical Group, Mountain View, California. Electronic address:
    The most recent reclassification of dermatoses of pregnancy includes polymorphic eruption of pregnancy, atopic eruption of pregnancy, and pemphigoid gestationis; intrahepatic cholestasis of pregnancy, strictly not a dermatosis, was included in specific dermatoses of pregnancy for working purposes. Another dermatosis, pustular psoriasis of pregnancy, could be included for similar reasons. The nomenclature of these pregnancy-specific eruptions has been revised several times, generating potential confusion among practitioners. Read More

    Retrospective analysis of pemphigoid gestationis in 32 Saudi patients - Clinicopathological features and a literature review.
    J Reprod Immunol 2016 08 4;116:42-5. Epub 2016 May 4.
    Dermatology residents, Saudi commission of health programs, Saudi Arabia.
    Background And Objectives: Pemphigoid gestationis (PG) is a rare autoimmune-mediated blistering disease that mainly affects pregnant women in their 2nd or 3rd trimester and immediate postpartum period. In addition to the clinical assessment, the diagnosis of PG is usually confirmed by histological and immunological studies. PG usually flares up at the time of delivery and spontaneously improves postpartum. Read More

    Utility of Direct Immunofluorescence Studies in Subclassification of Autoimmune Sub-Epidermal Bullous Diseases: A 2-Year Study in a Tertiary Care Hospital.
    Turk Patoloji Derg 2016 ;32(2):91-8
    Department of Pathology Bharati Vidyapeeth Deemed University Medical College and Hospital, SANGLI, INDIA.
    Objective: Sub-epidermal bullous disorders belong to immunobullous diseases which develop as a result of autoantibody action against epidermal basement membrane proteins. Clinically, they are tense bullae and do not rupture easily. They are classified into various forms based on histopathology and direct immunofluorescence patterns. Read More

    Clinical, demographic and immunopathological spectrum of subepidermal autoimmune bullous diseases at a tertiary center: A 1-year audit.
    Indian J Dermatol Venereol Leprol 2016 May-Jun;82(3):358
    Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Background: The subepidermal autoimmune bullous diseases are a subset of immunobullous diseases encountered less frequently in the Indian population. There is a paucity of data on the prevalence, demographic and clinicopathological spectrum of various subepidermal autoimmune bullous diseases from India.

    Aim: To determine the demographic and clinicopathological profile of subepidermal autoimmune bullous diseases in Indian patients, presenting to the Immunobullous Disease Clinic of Postgraduate Institute of Medical Education and Research, Chandigarh. Read More

    Concomitant pemphigus vulgaris and pemphigoid gestationis: a case report and review of the literature.
    Dermatol Online J 2016 Jan 15;22(1). Epub 2016 Jan 15.
    Baylor College of Medicine.
    Pemphigus and pemphigoid are two unique acquired immunobullous diseases with distinct clinical presentations, histological findings, and characteristic serology; they are rarely reported to coexist in the same patient. Herein we present a 29-year-old woman with a history of pemphigus vulgaris, diagnosed by histology and positive desmoglein-3 antibodies on ELISA. She presented to our clinic shortly after the delivery of her first child with tense vesicles and bullae on an erythematous base on her abdomen. Read More

    Bullous diseases: Kids are not just little people.
    Clin Dermatol 2015 Nov-Dec;33(6):644-56. Epub 2015 Sep 14.
    Departments of Dermatology & Pediatrics, Columbia University College of Physicians and Surgeons, 161 Fort Washington Avenue, 12th Floor, New York, NY, 10032, USA. Electronic address:
    Bullous diseases may be rare; however, this does not preclude the clinician from being familiar with their manifestations and treatment. After ruling out infection, genetically inherited blistering diseases are more likely to be the cause of blistering or erosions in the neonatal period, whereas immunobullous diseases are more common in adults. Published literature on immunobullous disorders reflects information gleaned from case reports and open-label case series; prospective studies and evidence-based treatments are limited. Read More

    Dermatological Diseases Associated with Pregnancy: Pemphigoid Gestationis, Polymorphic Eruption of Pregnancy, Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy.
    Dermatol Res Pract 2015 2;2015:979635. Epub 2015 Nov 2.
    Department of Dermatology, Bispebjerg Hospital, 2400 Copenhagen NV, Denmark ; Center for Medical Research Methodology, Department of Biomedical Sciences, University of Copenhagen, 2200 Copenhagen N, Denmark.
    Dermatoses unique to pregnancy are important to recognize for the clinician as they carry considerable morbidity for pregnant mothers and in some instances constitute a risk to the fetus. These diseases include pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. This review discusses the pathogenesis, clinical importance, and management of the dermatoses of pregnancy. Read More

    Pemphigoid Gestationis: A Case Report and Review of Management.
    J Drugs Dermatol 2015 Aug;14(8):904-7
    Background: Pemphigoid gestationis (PG) is a rare pruritic autoimmune dermatosis associated with several adverse fetal outcomes. An appropriate pharmacotherapy regimen reduces these risks while also providing symptomatic relief.

    Case: A woman in her second trimester presented with an intensely pruritic vesiculobullous rash diagnosed as PG. Read More

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