Pemphigoid Gestationis Research Articles

Polymorphic eruption of pregnancy

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. Histopathology is nonspecific, and immunofluorescence studies help differentiate PEP from pemphigoid gestationis. The pathogenesis of PEP remains elusive, and relevant theories are reviewed. There are no associated maternal or fetal risks, and treatment is largely symptomatic.

Pemphigoid Gestationis: A Rare Dermatosis of Pregnancy

Pemphigoid gestationis is a rare dermatosis of pregnancy that often goes unrecognized or misdiagnosed. It can lead to adverse fetal outcomes including preterm delivery, low fetal birth weight, and even neonatal pemphigoid gestationis1. As such, proper diagnosis and management is critical in reducing fetal risk.

Pemphigoid Gestationis: A Case Report and Review of Literature

Pemphigoid gestationis is a rare autoimmune blistering disease specific to pregnancy. It is characterized by pruritic, urticarial plaques with development of tense vesicles and bullae within the lesions. Recurrence with subsequent pregnancies are often more severe and may be associated with miscarriages, prematurity, low birth weight and rarely fetal death. We report this case of pemphigoid gestationis in view of the importance of early diagnosis and treatment for prevention of fetal risk.

Concomitant pemphigus vulgaris and pemphigoid gestationis: a case report and review of the literature

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. Biopsy was consistent with pemphigoid gestationis and direct immunofluorescence confirmed the diagnosis. To our knowledge, there are no other reported cases of pemphigoid gestationis occurring in a patient with pemphigus vulgaris.

Clinical and Histopathological Spectrum of Vesciculobullous Lesions of skin-A Study of 40 cases

Background: Vesiculobullous lesions of skin areheterogenous group of dermatological disorders and are not uncommon. These lesions may present with common clinical presentation as blister clinically, but histopathological features differ. Aims & Objective: This study is under taken to study the clinical and histopathological features of vesciculobullous disorders. Materials and Methods: A total 40 cases of clinically diagnosed blistering disorder were included, the punch biopsy of the lesion was taken. The biopsy was studied for histopathologicalfetures like level of blister formation, acantholysis, spongiosis, basement membrane zone distruction, presence or absence of inflammation. Results: In the present study amongst 40 cases, Pemphigus vulgaris was diagnosed in 12 (30%) cases and detected as most common type, followed by Bullous Pemphigoid in 11 (27.5%) cases. Herpes gestationis (Bullous Pemphigoid) was diagnosed in only one case. Commonly the blisters were seen on trunk, extremeties, axilla and groin. The lesions were slightly male preponderance in the present study. Most of the cases found between 41-70 years of age. Conclusion: The distribution of vesiculobullous lesions in relation to gender, age group, presenting site and histopathological features differ in each type. Histopathological examination gives valuable clue in the diagnosis of particular type of blistering disorder. Key words: Vesciculobullous, Blister, Histopathological, Clinical, Disorder

Utility of Direct Immunofluorescence Studies in Subclassification of Autoimmune Sub-Epidermal Bullous Diseases: A 2-Year Study in a Tertiary Care Hospital.

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. This study was undertaken to assess the incidence of various sub-epidermal bullous disorders and the utility of direct immunofluorescence in accurately classifying them, and to study the intensity and pattern of immunofluorescence in various sub-epidermal bullous disorders Material and Method: A 2-year study of 38 cases of sub-epidermal bullous disorders sent for direct immunofluorescence studies formed the study group. The specimens were processed as per standard protocols. The clinical details were obtained from case files and requisition sent for histopathological and direct immunofluorescence studies. Thirty-eight patients were diagnosed to have sub-epidermal bullous disorders over the period of 2 years. Twenty five cases of Bullous Pemphigoid, 5 cases of Dermatitis Herpetiformis, 3 cases of Linear IgA Bullous disorder, 2 cases of Bullous Systemic Lupus Erythematoses and 1 case each of Epidermolysis Bullosa Acquisita, Cicatricial Pemphigoid and Pemphigus Gestationis was diagnosed. Positive direct immunofluorescence was seen in 91.3% of the cases. Histopathology alone cannot classify sub-epidermal bullous disorders and direct immunofluorescence studies are mandatory in all of them. Bullous Pemphigoid needs to be distinguished from Epidermolysis Bullosa Acquisita which requires Salt split direct immunofluorescence studies. Dermatitis Herpetiformis, Bullous Systemic Lupus Erythematosus and Linear IgA Bullous disorder show more or less similar histological picture with neutrophilic microabscess. Direct immunofluorescence studies help in the majority of cases but further testing such as immunoblotting, immunoelectron microscopy or indirect immunofluorescence becomes essential in cases with overlapping features.

Pemphigoid gestationis – a rare disease encountered in pregnancy

Pemphigoid gestationis is a specific autoimmune disease of pregnancy. The onset occurs most commonly in the second or third trimester of pregnancy. Initially, the clinical picture is difficult to distinguish from polymorphic urticarial papules and plaques of pregnancy, urticarial plaques being present in both. Afterwards, the lesions form tense vesicles and bullae on an erythematous base. Direct immunofluorescence reveals C3 linear deposits, with or without IgG deposits along the basement membrane zone. Circulating antibodies are directed primarily against type XVII collagen (BP 180). The main fetal risks are preterm birth and fetal growth restriction. The treatment may be harmful for the fetus. Mild and moderate topical corticosteroids should be used. Systemic corticotherapy, azathioprine or dapsone should be administered in severe cases. Disease activity and treatment efficacy may be evaluated by measuring antibodies titer.

Eosinophil-associated dermatoses

Eosinophils induce tissue damage and organ dysfunction mainly through antigen-antibody reactions, release of toxic granules, mitochondrial DNA, and so on. Skin lesions induced by eosinophilia include erythema, plaques and nodules, usually with itching. Eosinophilia may manifest as an increase of eosinophil count in peripheral blood and eosinophilic infiltration of organs and tissues. Diseases associated with eosinophilia in both peripheral blood and the skin include hypereosinophilic syndrome, Churg-Strauss syndrome, Kimura′s disease, Wells′ syndrome, etc. Dermatoses associated with eosinophilia in peripheral blood alone include allergic cutaneous vasculitis, drug reaction with eosinophilia and systemic symptoms, etc, and those associated with eosinophilia in the skin alone include angiolymphoid hyperplasia with eosinophilia, pemphigoid gestationis, incontinentia pigmenti, etc. Key words: Eosinophilia; Skin diseases; Churg-Strauss syndrome; Inflammation; Immunity

Skin disease in pregnancy

Skin manifestations during pregnancy are common and diversified. This review will focus on the most important entities to be recognized by obstetricians. These are, on the one hand, physiological changes, where unnecessary investigations should be avoided, and on the other, the specific dermatoses of pregnancy. These develop electively in pregnancy, and they are currently grouped into three disorders: polymorphic eruption of pregnancy, atopic eczema of pregnancy, and pemphigoid gestationis. Arguments for recognition of these are presented including detection of anti-BP180 antibodies. Follow-up and treatment depend on the precise diagnosis. Risks in fetal prognosis may occur in rare pemphigoid gestationis cases.

Pemphigoïde de la grossesse et syndrome de Turner ; une association inhabituelle

The authors report an uncommon case of a pregnant woman with Turner syndrome (TS) whose pregnancy (with favorable outcome) was complicated by a pemphigoid gestationis in third trimester. This case points out that monitoring of these pregnancies is not limited to cardiac monitoring. Prevalence of auto-immune diseases is increased in the TS. Auto-immune dermatoses, such as pemphigoid gestationis, are one of the potential complications during pregnancy and should be known to practitioners.

Cyclosporine treatment in severe gestational pemphigoid.

Gestational pemphigoid, a rare autoimmune skin disease typically occurring during pregnancy, is caused by autoantibodies against collagen XVII. Clinically it is characterised by severe itching followed by erythematous and bullous lesions of the skin. Topical or oral glucocorticoids usually relieve symptoms, but in more severe cases systemic immunosuppressive treatments are needed. Data on immunosuppressive medication controlling gestational pemphigoid are sparse. We report 3 intractable cases of gestational pemphigoid treated with cyclosporine.

Dermatological Diseases Associated with Pregnancy: Pemphigoid Gestationis, Polymorphic Eruption of Pregnancy, Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy.

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.

Therapeutic Apheresis in Dermatological Diseases

Since the mid-1970s, membrane modules became available and plasma separation techniques have gained importance. Therapeutic apheresis (TA) has been successfully introduced in a variety of autoantibody-mediated diseases. In dermatology, TA is increasingly applied as a support treatment for severe and/or refractory autoimmune bullous diseases. All autoimmune disease are characterized by autoantibodies mediated against structural proteins of the skin and/or mucous membranes. Autoimmune blistering diseases have a high morbidity and mortality. These diseases include pemphigus vulgaris and bullous pemphigoid among others. Drug-induced pemphigus and other forms, such as dermatitis herpetiformis, herpes gestationis, scleroderma, pyoderma gangrenosum, epidermal necrolysis, Behcet disease, psoriasis vulgaris, Henoch-Schönlein purpura, and porphyria cutanea tarda, are also mentioned. Pathogenetical aspects of autoantibodies in these diseases are clearly demonstrated. TA has been shown to effectively remove the autoantibodies from blood and lead to rapid clinical improvement.

Pemphigoid gestationis.

ORIGINAL ARTICLE, p 120 Pemphigoid gestationis (PG) is a rare and self‐limiting immunobullous disorder of pregnancy, the exact pathogenesis of which has not been fully characterized. It is believed to result from a breakdown of the immunological tolerance of the mother to the fetoplacental unit resulting in autoantibodies to the main target antigen collagen XVII (BP180), which is presented to the maternal immune system by aberrantly expressed human leucocyte antigen (HLA) class II molecules in the placenta. Recent studies have demonstrated that these antibodies are mostly of the IgG4 subclass,1 unlike previous data supporting the role of IgG1 and IgG3 antibodies.2 These antibodies cross the placenta and cross‐react with collagen XVII in the maternal skin resulting in formation of immune complexes, activation of complement and inflammatory cell recruitment, with the release of inflammatory mediators resulting in blister formation on maternal skin. In most patients, antibodies target the extracellular noncollagenous (NC) 16A domain of BP180 antigen; however, in one study, the sera of nine of 15 patients with PG reacted with at least one additional antigenic site other than NC16A.3 In another study, about 10% of patients additionally showed antibodies to BP230 antigen, possibly due to the phenomenon of epitope spreading.4

妊娠性疱疹の 1 例

30 歳，女性。初産婦。妊娠 41 週 0 日に男児を正常分娩で出産し，児に異常はみられなかった。出産 2 日後より四肢を中心に紅斑と小水疱を伴う皮疹が出現し，徐々に拡大してきたため精査・加療目的に当院を紹介され受診した。初診時，特に足背・手背に瘙痒を伴う緊満性水疱が多発し，多形滲出性紅斑様の紅斑を認めた。血液検査所見では抗 BP180NC16a 抗体（以下抗 BP180 抗体）は 280 と高値，病理組織学的所見では表皮下水疱がみられ，水疱内にフィブリンを認めた。蛍光抗体直接法では，表皮基底膜部に IgG，C3 が線状に沈着していた。以上より自験例を妊娠性疱疹と診断した。プレドニゾロン（PSL）50mg/日から治療を開始し，皮疹は軽快した。現在 PSL 5 mg/日まで漸減しているが症状の再燃はみられていない。 本邦でこれまでに報告された産褥期に発症した妊娠性疱疹 9 例の抗 BP180 抗体の ELISA による測定値を検討したところ，産褥期発症例では出産前発症例に比較して低値であった。これに対し，自験例は産褥期発症例であるにもかかわらず高値を示した点が特異であると思われた。

Histopathological diagnosis of atopic eruption of pregnancy and polymorphic eruption of pregnancy: a study on 41 cases.

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.

Dermatoses de la grossesse

Pregnancy-specific dermatoses include polymorphic eruption of pregnancy, atopic eczema of pregnancy, and pemphigoid gestationis. Intrahepatic cholestasis of pregnancy and impetigo herpetiformis are not real pregnancy-specific dermatoses but they are important to know considering the fetal and maternal risks. Polymorphic eruption of pregnancy is a pruritic disease that usually occurs in primiparous women during the last trimester of pregnancy. Atopic eczema of pregnancy is still controversial as an entity covering conditions with eczematous lesions, prurigo, or folliculitis, and inconstantly associated with a personal history of atopy. Skin biopsy with direct immunofluorescence or search for serum anti-BPAg1 (180kD) NC16a antibodies is mandatory in pruritic dermatoses of pregnancy in order to rule out pemphigoid gestationis. Serum bile salts levels should be tested whenever a generalized pruritus develops during pregnancy in order to rule out intrahepatic cholestasis.

Gestational pemphigoid.

Gestational pemphigoid (pemphigoid gestationis, PG) is a rare autoimmune skin disorder occurring characteristically during pregnancy. Autoantibodies against placental BP180 (also known as BPAG2 or collagen XVII) cause damage to the skin basement membrane, resulting in severe itching and blistering rash over the body and the extremities. The diagnosis of PG is confirmed by immunofluorescence analysis of a skin biopsy, while serum levels of pemphigoid antigen BP180 antibody can be used to assess disease activity. PG with mild symptoms can be treated with topical corticosteroids, while oral corticosteroids are the mainstay in treatment of severe PG. PG usually flares up at the time of delivery, and resolves spontaneously shortly after. However, relapses in subsequent pregnancies are common. As PG has been linked to the risk of prematurity and fetal growth restriction, prenatal monitoring jointly by a dermatologist and an obstetrician is recommended. Mothers should also be informed of the potential risk of re-activation of the disease in subsequent pregnancies and during hormonal contraception.

Bullous pemphigoid: diagnosis and therapy

Bullous pemphigoid is the most common autoimmune bullous skin disease which occurs more often at higher age. Clinically it presents with tense blisters and eczematous lesions combined with severe pruritus. The pemphigoid-like diseases include mucous membrane pemphigoid, pemphigoid gestationis and linear IgA disease. Diagnosis is based on immunofluorescence microscopy and confirmatory tests (ELISA, immunoblotting). Classical bullous pemphigoid presents with IgG autoantibodies against BP180 and BP230. Treatment includes topical and systemic corticosteroids and adjuvant immunosuppressants.

Pemphigoid gestationis with prepartum flare

The Journal of DermatologyVolume 41, Issue 9 p. 850-851 Letter to the Editor Pemphigoid gestationis with prepartum flare Satoko Minakawa, Corresponding Author Satoko Minakawa Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanCorrespondence: Satoko Minakawa, M.D., Ph.D., or Daisuke Sawamura, M.D., Ph.D., Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori 036-8562, Japan. Email: [email protected] or [email protected]Search for more papers by this authorTakahide Kaneko, Takahide Kaneko Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorDaiki Rokunohe, Daiki Rokunohe Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorKoji Nakajima, Koji Nakajima Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorYasushi Matsuzaki, Yasushi Matsuzaki Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorHajime Nakano, Hajime Nakano Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorTakashi Hashimoto, Takashi Hashimoto Department of Dermatology, Kurume University School of Medicine, Kurume University Institute of Cutaneous Cell Biology, Kurume, JapanSearch for more papers by this authorDaisuke Sawamura, Corresponding Author Daisuke Sawamura Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanCorrespondence: Satoko Minakawa, M.D., Ph.D., or Daisuke Sawamura, M.D., Ph.D., Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori 036-8562, Japan. Email: [email protected] or [email protected]Search for more papers by this author Satoko Minakawa, Corresponding Author Satoko Minakawa Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanCorrespondence: Satoko Minakawa, M.D., Ph.D., or Daisuke Sawamura, M.D., Ph.D., Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori 036-8562, Japan. Email: [email protected] or [email protected]Search for more papers by this authorTakahide Kaneko, Takahide Kaneko Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorDaiki Rokunohe, Daiki Rokunohe Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorKoji Nakajima, Koji Nakajima Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorYasushi Matsuzaki, Yasushi Matsuzaki Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorHajime Nakano, Hajime Nakano Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanSearch for more papers by this authorTakashi Hashimoto, Takashi Hashimoto Department of Dermatology, Kurume University School of Medicine, Kurume University Institute of Cutaneous Cell Biology, Kurume, JapanSearch for more papers by this authorDaisuke Sawamura, Corresponding Author Daisuke Sawamura Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, JapanCorrespondence: Satoko Minakawa, M.D., Ph.D., or Daisuke Sawamura, M.D., Ph.D., Department of Dermatology, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori 036-8562, Japan. Email: [email protected] or [email protected]Search for more papers by this author First published: 16 July 2014 https://doi.org/10.1111/1346-8138.12576Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Volume41, Issue9September 2014Pages 850-851 RelatedInformation