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1.
Dermatol Clin. 2019 Apr;37(2):215-228. doi: 10.1016/j.det.2018.12.003. Epub 2019 Feb 14.

Treatment Update of Autoimmune Blistering Diseases.

Author information

1
Department of Dermatology, Rambam Health Care Campus, POB 9602, Haifa 31096, Israel.
2
Department of Dermatology, Wake Forest School of Medicine, Wake Forest University, 4618 Country Club Road, Winston-Salem, NC 27104, USA.
3
College of Medicine (Lake Nona), University of Central Florida, 6850 Lake Nona Boulevard, Orlando, FL 32827, USA.
4
Department of Medicine, University of Central Florida, Health Sciences Campus at Lake Nona, 2627 Northampton Avenue, Orlando, FL 32827-7408, USA. Electronic address: naveed.sami@ucf.edu.

Abstract

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.

KEYWORDS:

Autoimmune blistering diseases; Emerging; Rituximab; Treatment

PMID:
30850044
DOI:
10.1016/j.det.2018.12.003
[Indexed for MEDLINE]
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2.
G Ital Dermatol Venereol. 2019 Jun;154(3):256-262. doi: 10.23736/S0392-0488.18.06153-9. Epub 2018 Oct 29.

Autoimmune bullous diseases during pregnancy: insight into pathogenetic mechanisms and clinical features.

Author information

1
Section of Dermatology, Department of Medicine and Surgery, University of Parma, Parma, Italy.
2
Unit of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.
3
Department of Physiopathology and Transplantation, University of Milan, Milan, Italy.
4
Unit of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy - angelo.marzano@unimi.it.

Abstract

Pemphigoid gestationis (PG), also known as herpes gestationis, is the prototypic pregnancy-associated autoimmune bullous disease (AIBD), but also the other AIBDs, notably pemphigus vulgaris, may begin or exacerbate during pregnancy. Although the increase in concentration of T and B regulatory cells makes pregnancy a state of increased immunologic tolerance toward the semiallogeneic fetal antigens, a prevalent T helper (Th) 2 profile, that is reported to be associated with pregnancy, may cause exacerbation of pemphigus and AIBDs in general during this period. Active disease may lead to stillbirth, spontaneous abortion, preterm pregnancy, low birthweight, and neonatal pemphigus. PG is a rare AIBD usually starting during the third trimester of pregnancy and healing in the postpartum. It is due to the formation of autoantibodies directed against different epitopes of bullous pemphigoid (BP) 180 as a consequence of the aberrant expression of BP180 in the placental tissue of genetically predisposed women. PG is characterized by vesicles with herpetiform distribution, blisters and urticarial elements typically involving the periumbilical area and the distal portion of the upper limbs. Diagnosis is based on: 1) physical examination; 2) histopathological pattern consisting of a dermal inflammatory infiltrate rich in eosinophils; 3) direct immunofluorescence test demonstrating linear deposits of complement fraction 3 and immunoglobulin G along the basement membrane zone; 4) detection of circulating autoantibodies by means of indirect immunofluorescence or enzyme linked immunosorbent assay. Here, we provide an updated overview on the pathophysiologic mechanisms of pregnancy-associated or pregnancy-exacerbated AIBDs, focusing also on peculiar clinical features of these disorders.

PMID:
30375213
DOI:
10.23736/S0392-0488.18.06153-9
[Indexed for MEDLINE]
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3.
Dermatol Clin. 2018 Jul;36(3):259-265. doi: 10.1016/j.det.2018.02.012. Epub 2018 Apr 26.

Pruritus in Pregnancy and Its Management.

Author information

1
Division of Dermatology, Ohio State University College of Medicine, 540 Officenter Place, #240, Gahanna, OH 43230, USA. Electronic address: dermdocmab@aol.com.

Abstract

Pruritus in pregnancy can be a source of significant discomfort in the pregnant patient. Some cases are associated with pregnancy-specific dermatoses, although some patients experience a flare of a preexisting dermatosis. Severe pruritus may be a manifestation of a pregnancy-specific dermatosis associated with increased fetal risks and complications. Early accurate diagnosis and appropriate management are important. Examination often reveals important clinical findings, aiding accurate diagnosis. Pemphigoid gestationis often presents with periumbilical involvement, whereas polymorphic eruption of pregnancy spares the umbilicus and presents in the striae distensae. Intrahepatic cholestasis of pregnancy is associated with intense pruritus of the palms.

KEYWORDS:

Atopic eruption of pregnancy; Intrahepatic cholestasis of pregnancy; Pemphigoid gestationis; Polymorphic eruption of pregnancy; Pruritus

PMID:
29929597
DOI:
10.1016/j.det.2018.02.012
[Indexed for MEDLINE]
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4.
5.
J Dtsch Dermatol Ges. 2018 Jun;16(6):775-777. doi: 10.1111/ddg.13540.

Pemphigoid gestationis with lethal fetal malformation and postpartum persistence.

Author information

1
Department of Dermatology, Allergology and Dermatosurgery, HELIOS University Medical Center Wuppertal, University of Witten/Herdecke, Germany.
PMID:
29873913
DOI:
10.1111/ddg.13540
[Indexed for MEDLINE]
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6.
Acta Dermatovenerol Croat. 2018 Apr;26(1):71-72.

Urticarial Lesions in a Pregnant Woman.

Author information

1
Sergio Santos-Alarcon, MD, Department of Dermatology, , Hospital Universitario Doctor Peset, Avinguda Gaspar Aguilar Nº 90, 4601 Valencia, Spain; ssantosalarcon@gmail.com.

Abstract

Dear Editor, Gestational pemphigoid (GP) is a rare autoimmune bullous dermatosis in pregnancy. GP usually occurs during the second or third month of pregnancy. It clinically manifests as the development of either early-onset urticarial lesions or late-onset subepidermal blisters that may linger for weeks or even months. Herein we report the case of a 45-year-old woman with the distinctive clinical onset of GP. A forty-five-year-old woman, gravida I, para 0, at 27 weeks gestation, was referred for evaluation to our Department with an extensive pruritic eruption that had developed over the previous 7 days. The lesions had first appeared on the proximal thighs and extended progressively to the abdomen. On physical examination, numerous round urticarial plaques of approximately 1 cm in diameter were noted on her abdomen, involving the periumbilical area. Her thighs and back were also affected (Figures 1 and 2). The palms and soles were spared. No mucosal involvement was seen. The patient medical history was unremarkable, and she denied use of any other medications or herbal remedies at the time the symptoms started or since. No other symptoms but pruritus were referred. Laboratory studies, including complete blood cell count, coagulation tests, and renal and hepatic function were all normal. A punch biopsy was taken from an urticarial plaque and stained with hematoxylin and eosin. Histological examination found spongiosis in combination with an intraepidermal eosinophilic infiltrate, without the development of blisters (Figure 3). Direct immunofluorescence of perilesional skin showed linear deposition of complement (C3) along the basement membrane zone (Figure 4). Serum antibody titers for BP180NC16a were detected by enzyme-linked immunosorbent assay (ELISA). We established a diagnosis of gestational pemphigoid. Our patient was treated with systemic glucocorticoids, no blisters developed, and lesions cleared 8 weeks after delivery. The newborn girl did not developed neonatal gestational pemphigoid. Gestational pemphigoid, originally misnamed herpes gestationis, is a rare autoimmune bullous dermatosis in pregnancy. Single cases have been also described in patients with molar pregnancies and trophoblastic tumors (1). Its etiology is based in the development of autoantibodies against the fetoplacental unit, triggering an autoimmune response against both skin and amnion hemidesmosomal proteins, mainly BP180, but also BP230 and type VII collagen. An association with HLA-DR3 and HLA-DR4 has been described (2). GP usually occurs during the second or third month of pregnancy, but it may appear at any time during pregnancy or puerperium. In the vast majority of cases, symptoms alleviate a few weeks before delivery, but they reemerge at the time of delivery. Recurrences are frequent in following pregnancies, with an earlier onset and more severe symptoms, and may occur during subsequent menstruations or hormonal contraceptive use (1). GP clinically consists of the development of either early-onset urticarial lesions or late-onset subepidermal blisters that mat linger for weeks or even months. They generally appear on the abdomen, specifically in the periumbilical area, with posterior widespread extension to proximal limbs. Facial and mucosal lesions are uncommon (1). Histopathological studies are necessary to establish the diagnosis. These findings vary depending on the stage and severity of the disease and include subepidermal blisters, papillary dermal edema, eosinophilic spongiosis, and a polymorphous perivascular inflammatory cell infiltrate with a predominance of eosinophils. Direct immunofluorescence of perilesional skin shows a linear deposition of C3 along the basement membrane zone in all cases. IgG deposits can also be seen (3). These deposits are located within the lamina lucida and localized to the proximal part of anchoring filaments of the epidermal fragment of salt-split skin (4). Moreover, immunoblot and ELISA of the NC16a domain of BP180 RP are highly sensitive diagnostic methods in GP (5). The aim of treatment is to alleviate the pruritus and prevent formation of new blisters. Topical corticosteroids and oral antihistamines may be used in mild cases. Systemic corticosteroids are the treatment of choice in moderate to severe cases. Other treatments that have been used are cyclophosphamide, dapsone, gold, methotrexate, and plasmapheresis (5).

PMID:
29782306
[Indexed for MEDLINE]
7.
BMJ Case Rep. 2018 Apr 7;2018. pii: bcr-2018-224346. doi: 10.1136/bcr-2018-224346.

Pemphigoid gestationis successfully treated with intravenous immunoglobulin.

Author information

1
Department of Dermatovenereology, Hospital de Braga, Braga, Portugal.
2
Department of Gynecology and Obstetrics, Hospital de Braga, Braga, Portugal.
3
Department of Surgical Pathology, Hospital de Braga, Braga, Portugal.

Abstract

Pemphigoid gestationis (PG), also known as herpes gestationis, 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.1Although the ethiopathogeny of PG is not fully clarified, most patients develop antibodies against a 180 kDa transmembrane hemidesmosomal protein (BP180; BPAG2; collagen XVII).2 PG has a strong association with human leucocyte antigens DR3 and DR4.3We report a case of a 29-year-old female patient with PG successfully treated with intravenous immunoglobulin.

KEYWORDS:

dermatology; obstetrics and gynaecology; obstetrics, gynaecology and fertility

PMID:
29627782
DOI:
10.1136/bcr-2018-224346
[Indexed for MEDLINE]
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8.
J Dermatolog Treat. 2018 Dec;29(8):815-818. doi: 10.1080/09546634.2018.1459034. Epub 2018 May 7.

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

Author information

1
a Department of Dermatology , Wake Forest School of Medicine , Winston-Salem , NC , USA.

Abstract

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. Mucosal lesions and facial involvement are rare. In this article, we are discussing three cases of PG. We will discuss clinical-pathological features and management.

KEYWORDS:

Pemphigoid gestationis; collagen XVII; pregnancy; pruritus

PMID:
29620424
DOI:
10.1080/09546634.2018.1459034
[Indexed for MEDLINE]
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9.
J Cutan Med Surg. 2018 Jul/Aug;22(4):435-438. doi: 10.1177/1203475418760459. Epub 2018 Feb 28.

Pemphigoid Gestationis: A Case Presentation.

Author information

1
1 Department of Obstetrics and Gynecology, University of Toronto, Toronto, ON, Canada.
2
2 Department of Laboratory Medicine, St Michael's Hospital, and the Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.

KEYWORDS:

case report; dermatoses; pemphigoid gestationis; pregnancy; pruritus in pregnancy

PMID:
29486593
DOI:
10.1177/1203475418760459
[Indexed for MEDLINE]
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10.
Int J Dermatol. 2018 May;57(5):534-540. doi: 10.1111/ijd.13925. Epub 2018 Feb 23.

A subset of patients with pemphigoid (herpes) gestationis has serological evidence of celiac disease.

Author information

1
Immunodermatology Laboratory, Department of Dermatology, School of Medicine, University of Utah, Salt Lake City, UT, USA.

Abstract

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.

CONCLUSIONS:

A subset of patients with serological findings of PG also has serological evidence of CD, which may have implications in the etiopathogenesis of PG and which reveals important information about the mother's, and possibly her infant's, health.

PMID:
29473148
DOI:
10.1111/ijd.13925
[Indexed for MEDLINE]
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11.
JAMA Dermatol. 2018 Apr 1;154(4):484-486. doi: 10.1001/jamadermatol.2017.5539.

Anhydramnios in Patients With Pemphigoid Gestationis.

Author information

1
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
2
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
3
Department of Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
PMID:
29365022
DOI:
10.1001/jamadermatol.2017.5539
[Indexed for MEDLINE]
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13.
Australas J Dermatol. 2018 May;59(2):e123-e126. doi: 10.1111/ajd.12656. Epub 2017 May 19.

Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder.

Author information

1
Department of Neurology, Mohammed V. Rabat Military Hospital, Rabat, Morocco.
2
Department of Cardiology, Mohammed V. Rabat Military Hospital, Rabat, Morocco.
3
Department of Pathology, Ibn Sina Hospital, Rabat, Morocco.

Abstract

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.

KEYWORDS:

cardiomyopathy; encephalitis; gestationis; pemphigoid

PMID:
28524368
DOI:
10.1111/ajd.12656
[Indexed for MEDLINE]
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14.
Br J Dermatol. 2017 Apr;176(4):e36. doi: 10.1111/bjd.15325.

Image Gallery: Pemphigoid gestationis mimicking a gyrate erythema.

Author information

1
Department of Dermatology, Dankook University College of Medicine, San 16-5, Anseo dong, Cheonan, Chungnam, Republic of Korea.
PMID:
28418130
DOI:
10.1111/bjd.15325
[Indexed for MEDLINE]
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15.
16.
J Obstet Gynaecol. 2017 Aug;37(6):824-826. doi: 10.1080/01443615.2017.1305334. Epub 2017 Apr 11.

Early flare-up of severe Herpes gestationis (Pemphigoid gestationis) and successfull prolonged treatment: A case report.

Author information

1
a Department of Obstetrics and Gynecology , University Hospital Martin and Jessenius Faculty of Medicine, Comenius University , Martin , Slovakia.
2
b Biomedical Center Martin, Jessenius Faculty of Medicine , Comenius University , Martin , Slovakia.
3
c Dermatovenerologic Clinic , University Hospital Martin and Jessenius Faculty of Medicine, Comenius University , Martin , Slovakia.
4
d Department of Pathological Anatomy , University Hospital Martin and Jessenius Faculty of Medicine, Comenius University , Martin , Slovakia.
PMID:
28399702
DOI:
10.1080/01443615.2017.1305334
[Indexed for MEDLINE]
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18.
J Am Acad Dermatol. 2017 Mar;76(3):560-562. doi: 10.1016/j.jaad.2016.09.030.

Sensitivity and specificity of BP180 NC16A enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis.

Author information

1
Department of Dermatology, INSERM U1234, Rouen University Hospital, University of Rouen, France.
2
Department of Immunology, Rouen University Hospital, University of Rouen, France.
3
Department of Gynecology and Obstetrics, Rouen University Hospital, University of Rouen, France.
4
Department of Dermatology, INSERM U1234, Rouen University Hospital, University of Rouen, France. Electronic address: Pascal.Joly@chu-rouen.fr.
PMID:
28212762
DOI:
10.1016/j.jaad.2016.09.030
[Indexed for MEDLINE]
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19.
Int J Dermatol. 2017 Mar;56(3):327-329. doi: 10.1111/ijd.13536. Epub 2017 Jan 11.

The see-saw of immune dysregulation association of diverse organ autoimmune conditions in a woman. Evolution over twenty-seven years.

Author information

1
Chairs of Dermatology and Immunology, Instituto Nacional de Dermatología (now Instituto de Biomedicina,"Jacinto Convit"), Vargas School of Medicine, Central University of Venezuela, Caracas, Venezuela.
PMID:
28078771
DOI:
10.1111/ijd.13536
[Indexed for MEDLINE]
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20.
Hautarzt. 2017 Feb;68(2):87-94. doi: 10.1007/s00105-016-3922-z.

[Specific dermatoses of pregnancy].

[Article in German]

Author information

1
Hautarztpraxis, Mozartgasse 4, 8010, Graz, Österreich. ambros-rudolph@mozartpraxis.at.
2
Hautklinik, Universitätsklinikum Erlangen, Ulmenweg 18, 91054, Erlangen, Deutschland.

Abstract

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.

KEYWORDS:

Atopic eruption of pregnancy; Classification; Intrahepatic cholestasis of pregnancy; Pemphigoid gestationis; Polymorphic eruption of pregnancy

PMID:
28074212
DOI:
10.1007/s00105-016-3922-z
[Indexed for MEDLINE]
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