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

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.

Pemphigoid gestationis in a female with progressive facial hemiatrophy: microchimerism as a speculative shared background is disputable.

The commonest source of naturally acquired microchimerism, i.e. small numbers of foreign cells within the organism, is two-way mother-fetus transplacental trafficking during pregnancy. Here, the first report on coexistence of pregnancy-associated pemphigoid gestationis (PG) and progressive facial hemiatrophy, a form of "en coup de sabre" morphea, is presented. HE histopathology (eosinophil-rich subepidermal infiltration, inverted teardrop sign), direct immunofluorescence (linear IgG1, but not IgG4, deposits along the dermal-epidermal junction) and ELISA (elevated levels of serum and blister fluid IgG autoantibodies to BP180) corroborated the PG diagnosis. Microchimerism as a speculative shared background of those two rare autoimmune diseases is disputable.

[Rituximab (Mabthera) for treatment of rare autoimmune bullous skin disorders].

Autoimmune bullous skin disorders are characterized by a severe and potentially lethal course and may require aggressive long-term treatment with systemic corticosteroids and other immunosuppressive drugs, which can lead to serious adverse events. Recently, anti-CD20 antibody, Rituximab, was reported to be beneficial as an adjuvant therapy in these diseases. Herein, we present 2 case reports of patients suffering from resistant rare diseases from the aforementioned spectrum: linear IgA dermatosis and Pemphigoid gestationis. The patients were successfully treated with Rituximab (Mabthera). This is one of the first reports of this kind of treatment for these rare life-threatening diseases. These case reports emphasize the role of Rituximab as a crisis therapy in autoimmune blistering diseases.

Pregnant women have increased incidence of IgE autoantibodies reactive with the skin and placental antigen BP180 (type XVII collagen).

BP180 (type XVII collagen) is a transmembrane protein expressed in a variety of cell types. It is also the target of autoantibodies in cutaneous autoimmune disease including bullous pemphigoid and pemphigoid gestationis, a disease unique to pregnancy. The purpose of this study was to determine the prevalence and specificity of cutaneous autoantibodies in a cohort of pregnant women. De-identified sera were collected from pregnant women (n=299) and from non-pregnant controls (n=134). Sera were analyzed by ELISA for the presence of IgG and IgE autoantibodies directed against several cutaneous autoantigens. IgE antibodies against the NC16A domain of BP180 were detected in 7.7% of pregnant women, compared to 2.2% of healthy controls (p=0.01). No increase in total or cutaneous autoantigen specific IgG was seen. Total serum IgE was within the normal range. Full-length BP180 was detected by western immunoblot in epidermal, keratinocyte, placental and cytotrophoblast (CTB) cell lysates. Furthermore, flow cytometry and indirect immunofluorescence confirmed the expression of BP180 on the surface of cultured CTBs. Finally, it was demonstrated that IgE antibodies in the pregnancy sera labeled not only cultured CTBs, but also the placental amnion and cutaneous basement membrane zone using indirect immunofluorescence. We conclude that some pregnant women develop antibodies specific for BP180, and that these autoantibodies are capable of binding both CTB and the placental amnion, potentially affecting placental function.

Measurement of pruritus in a Chinese woman with pemphigoid gestationis using a wrist movement detector.

Pemphigoid gestationis (PG) is a rare itching bullous disease of pregnancy and the postpartum period. We describe the use of a new methodology for measuring the nature and intensity of itching in a 21-year-old woman with pemphigoid gestationis. At 19 weeks gestation, she developed an itchy rash over the limbs, which spread to the trunk. Blisters then appeared on the hands which subsequently also involved the feet. Intense nocturnal wrist activities in this patient, measured with a wrist monitor and defined as average acceleration in the early hours of sleep, were even higher than that in patients with severe eczema, with an average value of 181.00 +/- 43.49 (mean +/- standard error) g/min for the first three hours, versus 84.47 +/- 8.53 g/min for the group of 24 eczema subjects. Most wrist activities were slower movements at 0 to 1 Hz. This is in striking contrast to the scratching activities at 0 to 3 Hz in eczema subjects. There have been no therapeutic trials for PG. Topical steroid and oral antihistamines are usually ineffective, but worked in our patient. We also discuss the potential application of the monitor in assessing the nature of various dermatological or systemic itching disorders.

A case report of neonatal convulsions due to maternal herpes gestationis.

Herpes gestationis is a rarely seen autoimmune vesiculobullous and pruritic dermatosis of pregnancy. Neonatal morbidity associated with maternal herpes gestationis is controversial. The authors report the first case of neonatal convulsion with abnormal electroencephalography findings and transient vesicular eruption due to maternal herpes gestationis.

Immunological aspects of pregnancy.

Alterations to the immune status of the pregnant woman are necessary to allow mothers to tolerate genetically different fetal tissues during pregnancy. These alterations lead to impaired cell-mediated immunity with increased susceptibility to certain infections such as tuberculosis. During pregnancy, the maternal immune system also shows a relative bias toward T helper type 2 immunity. Several inflammatory dermatoses are either unique to pregnancy or altered by the pregnant state. Immunologists are now beginning to understand the various factors that contribute to the maternal immune tolerance and, in particular, the role of classic (human leukocyte antigen [HLA]-A, HLA-B, HLA-C, and HLA-D) and non-classic (HLA-E, HLA-F, and HLA-G) major histocompatability antigens in this process. Human leukocyte antigen-G, in particular, seems to be important in protecting HLA mismatched tissue from the innate immune system, and investigation of HLA-G expression may help to explain how pregnancy affects inflammatory skin disease. Immunologists are now beginning to understand the alterations to the immune status of the pregnant woman that are necessary to allow mothers to tolerate genetically different fetal tissues during pregnancy. These alterations may help to explain how pregnancy effects inflammatory skin disease.

Herpes gestationis (Pemphigoid gestationis).

Herpes gestationis, coined by Milton in 1872, or gestational pemphigoid is the most clearly characterized dermatosis of pregnancy. It is a rare vesiculo-bullous eruption that develops during the last trimester or even postpartum and creates severe pruritus. Its etiology is unknown, but it is considered as an autoimmune-mediated dermatosis closely related to the pemphigoid group. Herpes gestationis is associated with a positive C3 deposition along the base of the epidermis in salt-split skin, with increased frequency of HLA-DR3 and also the combination DR3 and DR4. It has a high risk of prematurity and disappears in the postpartum period within weeks or months.

Dermatosis del embarazo / Dermatosis of pregnancy

Se hace una revisión sobre las diversas dermatosis en la mujer embarazada. Se enfatiza sobre sus características clínicas, hallazgos de laboratorio; datos histopatológicos, de inmunofluorescencia; sobre su tratamiento, pronóstico de riesgo materno-fetal y diagnóstico diferencial

Herpes gestacional: presentación de un caso y revisión de la literatura / Herpes gestationis. A case presentation and literature review

El herpes gestacional (HG) es una enfermedad autoinmune de la piel de tipo ampuloso que se relaciona exclusivamente con la gestación, es de etilogía desconocida y se presenta un caso de 300 a 50000 embarazos. El diagnóstico se establece clínicamente pero debe confirmarse por medio de inmunohistopatología. El tratamiento consiste en medidas locales y corticoesteroides sistémicos. Aunque el pronóstico materno es excelente existen controversias en cuando al resultado perinatal. Se presenta un caso de HG y se hace revisión de la literatura

Herpes gestationis--bullae from hormones that cause increased extracellular matrix (autoantibodies to epidermal basement membrane as a secondary phenomenon).

Herpes gestationis is a bullous skin disease clearly secondary to the hormones of pregnancy and other hormonal influences. It is the result multiple hormones. No one hormone is specific. The hormones increase ground substance viscosity in the skin and this induces edema and bullae formation. Only 10 to 20% of the patients demonstrate IgG formation by indirect immunofluorescence. By direct immunofluorescent studies 30 to 40% of the patients demonstrate deposition of IgG in the basement membrane zone. Other variations of immune factors are present in most cases but they are weak by titers usually present in most so called autoimmune disorders. Many bullous diseases of the skin, including herpes gestationis, are considered autoimmune. It is known that trauma to cells and tissues can induce autoantibodies to form. It is proposed that autoantibodies can form as a secondary phenomenon in bullous skin disorders. The authors who have proposed an autoimmune etiology of herpes gestationis do not attempt to explain the clear association of the disease with the hormones of pregnancy, other female hormones, chorio-carcinoma, or hydatidiform mole.

Herpes gestationis.

Only 14 years have elapsed since immunofluorescence techniques revised our concept of herpes gestationis. As our inquiry broadens, the answers seem farther away. Yet much knowledge has been gained regarding predisposition and the pathomechanism of this elusive disease. I offer a review of historical context and an update on our current confusions regarding this rare autoimmune dermatosis of pregnancy.

[Herpes gestationis (author's transl)]. / Herpes gestationis.

Herpes gestationis is a rare blistering disease of pregnancy and the puerperium which is difficult to differentiate from other bullous eruptions of pregnancy by clinical and histopathological criteria only. Caracteristic immunopathological features permit the exact classification of this disease. This is a report on three typical cases of herpes gestationis. The clinical, histopathological and immunopathological findings of the disease as well as treatment and maternal and fetal risks are discussed.