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PubMedDate not supplied

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.

Female↗
PubMed1992

Secondary autoimmune diseases in herpes gestationis (pemphigoid gestationis).

BACKGROUND: Herpes gestationis (HG) is an autoimmune disease of the skin that occurs exclusively in association with pregnancy (or trophoblastic disease). It is associated with the HLA-DR3 and -DR4 antigens that are also associated with several other autoimmune diseases. HG has previously been reported in association with Graves' disease. OBJECTIVE: Our purpose was to determine the frequency of other autoimmune disease(s) in patients with a history of HG. METHODS: Seventy-five patients with a history of HG were studied for the frequency of other autoimmune diseases. RESULTS: We found an increased frequency of Graves' disease in patients with a history of HG. Those with HG have an increased risk for the development of other autoantibodies. There is an increased frequency of autoimmune diseases in the family members of patients with HG. CONCLUSION: Secondary autoimmune disease in HG is unusual, but does occur. The most frequent is Graves' disease.

Autoantibodies↗
PubMed1993

Anti-HLA antibodies in pemphigoid gestationis (herpes gestationis).

Pemphigoid gestationis (PG; herpes gestationis) is a rare autoimmune disease associated with pregnancy, currently defined by the presence of complement deposition along the cutaneous basement membrane zone. It is known to be associated with HLA-DR3 and DR4, and an increase in anti-HLA antibodies in those with a history of PG has been reported. We have studied 39 patients with an immunofluorescence-confirmed diagnosis of PG for the presence and specificity of anti-HLA antibodies. Anti-HLA antibodies were found in all 39 patients. Specificity was against class I antigens in 98% (controls 10%; P < 0.001) and class II antigens in 25% (controls 8.5%; P < 0.001). Almost all anti-HLA antibodies were cytotoxic. The universal presence of anti-HLA antibodies in PG suggests that they may develop coincidently with antibasement membrane antibodies, and may reflect a common immunological event.

Adult↗
PubMed1995

Class II MHC typing in pemphigoid gestationis.

Pemphigoid gestationis (PG) is a rare, autoimmune skin disease associated with pregnancy or the immediate post-partum period, previously shown to be associated with the HLA class II antigens DR3 and DR4. Advances in molecular analytical techniques now allow the identification of HLA alleles previously difficult to define by serological assays. Unsuspected polymorphism within the HLA-DR3 and DR4 classes can, therefore, be identified. The aim of our study was to apply these newer techniques to the question of genetic predisposition in PG by re-evaluating the association with DR3 and by studying a possible link with DQ. We have investigated by restriction fragment length polymorphism, the DQA, and by sequence specified oligonucleotide probing the DQB and DRB1 (HLA DR) specificities of 41 women with immunofluorescence-confirmed PG. The principal finding of this study is that there is an association between PG and DRB1*0301 (DR3) and DRB1*0401/040X (DR4). Although there is also an increase (P = 0.06) in the concurrent presence of both antigens, this appears to be due to the association with either antigen alone. We also found an increase in the frequency of DQA1*2 (P = 0.016 vs. control) and a decrease in frequency of DQB1*0201 (P = 0.022 vs. controls) and DQB1*0602 (P = 0.026 vs. controls).

Case-Control Studies↗
PubMed2002

Successful treatment with doxycycline and nicotinamide of two cases of persistent pemphigoid gestationis.

Pemphigoid gestationis (PG) is a rare dermal-epidermal autoimmune bullous disease of pregnancy and postpartum, which relapses more seriously and earlier during following pregnancies. PG also occurs in association with trophoblastic tumours or oral contraceptive treatment. The term 'persistent PG' represents the cases where active disease persists for months to many years after delivery. Four cases of persistent PG have been reported to date in the literature. So far, systemic cortico-steroids have been the main PG therapy and the use of cyclophosphamide, dapsone, pyridoxine, methotrexate, plasmapheresis or ritodrine has also been reported, with contradictory results. In this paper are described two patients with persistent PG who were successfully treated with doxycycline (200 mg/day) and nicotinamide (500 mg/day), a treatment that was demonstrated to be safe and efficacious in bullous pemphigoid.

Adult↗
PubMed1996

Conversion of pemphigoid gestationis to bullous pemphigoid--two refractory cases highlighting this association.

Pemphigoid gestationis and bullous pemphigoid are autoimmune diseases characterized by subepidermal blisters and antibodies against the hemidesmosomal antigens: BPAG1 and BPAG2. Clinical histological and immunological similarities between pemphigoid gestationis and bullous pemphigoid suggest that they may have common pathogenetic determinants. We report two patients who presented initially with clinico-pathological features characteristic of pemphigoid gestationis but who subsequently evolved into bullous pemphigoid.

Adult↗
PubMed2005

Usefulness of BP180 NC16a enzyme-linked immunosorbent assay in the serodiagnosis of pemphigoid gestationis and in differentiating between pemphigoid gestationis and pruritic urticarial papules and plaques of pregnancy.

BACKGROUND: Pemphigoid gestationis (PG) is a rare pregnancy-associated subepidermal immunobullous disease that targets hemidesmosomal proteins, particularly BP180. Clinically, PG can resemble the eruption known as polymorphic urticarial papules and plaques of pregnancy (PUPPP), and accurate differentiation between these 2 pruritic pregnancy dermatoses has important implications for fetal and maternal prognoses. Results of epitope mapping studies show that IgG autoantibodies in up to 90% of PG serum samples target the well-defined membrane-proximal NC16a domain of BP180. OBJECTIVE: To examine the usefulness of a commercially available NC16a domain enzyme-linked immunosorbent assay in the serodiagnosis of PG and in the differentiation of PG from PUPPP. PARTICIPANTS: A total of 412 women consisting of pretreatment patients with PG (n = 82), patients with PUPPP (n = 164), and age- and sex-matched controls (n = 166). METHODS: All serum samples were assayed in duplicate. Receiver operating characteristic analyses were performed to determine a cutoff value for the diagnosis of PG and for differentiation from PUPPP and controls. RESULTS: A cutoff value of 10 enzyme-linked immunosorbent assay units was associated with specificity and sensitivity of 96%. CONCLUSIONS: The NC16a enzyme-linked immunosorbent assay is highly sensitive and highly specific in differentiating PG from PUPPP, and it is potentially a valuable tool in the serodiagnosis of PG.

Adult↗
PubMed2006

IgG4 as the predominant IgG subclass in pemphigoides gestationis.

BACKGROUND: Pemphigoides gestationis (PG) is a blistering disorder of pregnancy caused by antibodies against basement membrane proteins. They are directed against the 180 kD bullous pemphigoid antigen (BPAg2), towards the epitopes within the NC 16A domain. There are many similarities between pemphigoid gestationis and bullous pemphigoid (BP), but the literature so far indicated different immunofluorescence results in regards with C3 and IgG, and IgG subclasses (IgG4 vs. IgG1). METHODS: We evaluated staining patterns and IgG subclasses, as well as C5b-9 membrane attack complex (MAC) in 10 pregnant patients with PG, using sandwich double antibody immunofluorescence (SDAI) and direct immunofluorescence (DIF). RESULTS: All ten specimens stained with C3 by DIF, but only five had trace amount of IgG reactants by this method. By SDAI, 100% were positive for the IgG4 and C5b-9 MAC, 70% for IgG2, 50% for IgG1, and 40% for IgG3. CONCLUSION: IgG4 was the predominant IgG subtype identified. This finding has not been reported for PG, but it mimics results reported for BP. One explanation is prolonged disease course, as well as blocking of antigenic domains by IgG4. Understanding this completely will help develop therapies and prevention strategies for immunobullous and other autoimmune diseases, and perhaps aid in an exact classification.

Complement Membrane Attack Complex↗
PubMed2006

The intracellular and extracellular domains of BP180 antigen comprise novel epitopes targeted by pemphigoid gestationis autoantibodies.

Pemphigoid gestationis (PG) is an autoimmune sub-epidermal bullous dermatosis of pregnancy associated with circulating autoantibodies targeting the extracellular non-collagenous (NC) 16A domain of bullous pemphigoid (BP) 180 antigen. In order to determine whether BP180 regions other than NC16A are recognized by PG autoantibodies, we have analyzed the reactivity of 15 PG patient sera against several BP180 antigenic sites by sensitive methods such as immunological screening and ELISA. Most PG sera tested (13 of 15) reacted with an epitope (amino acid 508-541) mapped in the NC16A domain. Of note, nine of 15 PG patient sera reacted with at least one additional antigenic site other than NC16A. Specifically, two epitopes in the BP180 extracellular domain and five epitopes in the intracellular one were recognized by three and seven PG sera, respectively. In addition, a representative intracellular epitope was recognized by PG autoantibodies as a portion of BP180 antigen both in denaturating and native conditions. Finally, reactivity against epitopes additional to NC16A was also detected at an early stage of the disease. The identification and characterization of hitherto unrecognized epitopes targeted by PG patient autoantibodies provide novel insights into the pathophysiology of humoral immune response to BP180 in PG.

Adult↗
PubMed2006

Immunoadsorption against two distinct epitopes on human type XVII collagen abolishes dermal-epidermal separation induced in vitro by autoantibodies from pemphigoid gestationis patients.

Pemphigoid gestationis (PG) is a subepidermal autoimmune blistering disease characterized by self-reactive T and B cells specific for the transmembrane hemidesmosomal protein type XVII collagen/BP180. Major T and B cell epitopes are located within the immunodominant 16th non-collagenous domain A (NC16A) of type XVII collagen. The aim of the present study was to map the pathogenically relevant epitopes targeted by blister-inducing patients' autoantibodies. For this purpose, we used an in vitro model of autoantibody-induced leukocyte-dependent dermal-epidermal separation. Pre-adsorption against a recombinant form of the NC16A region abolished the blister-inducing potential of autoantibodies from all PG patients. Using overlapping synthetic peptides, we demonstrated that PG autoantibodies bind to two defined epitopes within the NC16A region (aa 500-514 and aa 511-523). Importantly, pre-adsorption using an affinity matrix containing these epitopes completely abolished dermal-epidermal separation induced by PG autoantibodies. This study identifies the epitopes relevant for blister induction in PG and should facilitate the development of an antigen-specific immunoadsorption therapy for this disease.

Antibody Specificity↗
PubMed1990

Antigen-presenting cells in the skin and placenta in pemphigoid gestationis.

In pemphigoid gestationis (PG), one of the major initiating events is the aberrant expression of the class II molecules of the major histocompatibility complex in the placenta. We used a panel of 13 monoclonal antibodies to investigate the phenotype of the MHC class II positive antigen-presenting cells (APC) in the skin and placenta in PG. In the skin, the APC show reactivity with a variety of macrophage markers and the CDI marker of Langerhans cells. By contrast, the MHC class II positive cells in the placenta showed no reactivity with macrophage or Langerhans cell markers, but were the cytokeratin-positive trophoblast or vimentin-positive stromal cells.

Antigen-Presenting Cells↗
PubMed2003

The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis.

BACKGROUND: Pemphigoid gestationis (PG), also known as herpes gestationis, is a rare autoantibody-mediated bullous disease, usually associated with pregnancy and the postpartum period. However, infiltrating cells have recently been suggested to also contribute to the pathogenesis of cutaneous lesions. OBJECTIVES: To evaluate the immunophenotype of T cells infiltrating the PG lesional skin and their prevalent cutaneous cytokine expression, as well as the presence and distribution of mast cells, eosinophils and neutrophils. Methods We performed an immunohistochemical study with a large panel of monoclonal antibodies to CD3, CD4, CD8, HLA-DR, CD25, myeloperoxidase, tryptase, eosinophil cationic protein EG2, human interleukin (IL)-2, -4, -5, -8, interferon (IFN)-gamma, and granulocyte-macrophage colony-stimulating factor using the alkaline phosphatase-antialkaline phosphatase procedure on lesional skin of seven patients with PG. Skin from four subjects with pruritic urticarial papules and plaques of pregnancy and three additional healthy donors were used as controls. RESULTS: The findings indicate that there is a T-cell population with a prevalent T-helper (Th) 2 phenotype in the lesional skin of PG subjects. We also found a number of eosinophils and neutrophils with clear signs of activation. CONCLUSIONS: These data suggest that an inflammatory infiltrate is involved in the production of PG bullous lesions. In particular, we assume that the Th2 cells might be implicated in the very early stages of autoimmune response and may exercise a broad influence in blister formation in this disease.

Adult↗
PubMed2000

Pemphigoid gestationis: a review.

Pemphigoid gestationis is a rare autoimmune vesiculobullous skin disease closely related to the pemphigoid group of blistering disorders. It is unique in that it is most frequently associated with pregnancy. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of the third component of complement along the basement membrane zone of perilesional skin. Abnormal expression of major histocompatibility complex class II molecules in the placenta may trigger the initiating immunologic event in this organ, which is followed by cross-reactivity with an antigen present in the skin and the characteristic cutaneous signs of the disease. The immunogenetics of this disorder are interesting. A role for the involvement of major histocompatibility complex class II antigens seems likely because of the association with human leukocyte antigens DR3 and DR4. Major histocompatibility complex class III associations have also been observed, as has a high frequency of anti-human leukocyte antigen antibodies. We review the current understanding of the molecular biologic and immunogenetic properties of this disease and discuss treatment and potential impact on the fetus.

Diagnosis, Differential↗
PubMed2004

[Pemphigoid gestationis: a study of 20 cases].

INTRODUCTION: Pemphigoid gestationis is a bullous autoimmune sub-epidermal dermatosis, occurring during pregnancy and/or postpartum. The objective of our study was to define the epidemio-clinical profile, the histopathological and immunopathological features, the treatment and the course of pemphigoid gestationis in Tunisian patients. PATIENTS AND METHODS: This was a retrospective study concerning the cases of pemphigoid gestationis recorded between 1989 and 2003 in the dermatology department in La Rabta Hospital in Tunis. The patients were included according to clinical, histopathological and immunopathological criteria. RESULTS: We retained 20 patients: 15 multiparae and 5 primiparae. The average age at onset was 29. The first clinical signs appeared in the 3rd trimester in 60 p. 100 of all cases. Clinically, the urticarial patches were noticed in 90 p. 100 of all cases and blisters in 65 p. 100 of the cases. The eruption was located mainly on the trunk and the limbs. The face was affected in 7 cases, the mucous membranes in 3 cases, the palms in 2 cases and the soles in 1 case. Cutaneous histologic examination revealed a sub-epidermal blister in 11 cases and a lymphohistiocyte infiltrate in all cases. Direct immunofluorescence showed a linear deposition of the third component of the complement along the basement membrane zone in all cases. Fetal prognosis was assessed in 13 cases: 1 fetal death, 1 still-born, 3 miscarriages, 1 anencephaly and 2 cases of transit bullous affection in the new-born. DISCUSSION: Our results are similar to those in the literature, but with some particular aspects: the late onset of the pemphigoid gestationis in the course of the 3rd trimester of pregnancy, the frequent involvement of the face and the mucous membranes and the absence of the two main fetal risks: prematurity and hypotrophy. Moreover, we underline the efficiency of topical class I corticosteroids in the treatment of pemphigoid gestationis.

Administration, Topical↗
PubMed2006

Clinical experience in pemphigoid gestationis: report of 10 cases.

BACKGROUND: Pemphigoid gestationis is a rare autoimmune blistering disease that occurs during pregnancy. OBJECTIVE: This study reviewed our clinical experience with pemphigoid gestationis. METHODS: We reviewed medical records of 10 patients with pemphigoid gestationis seen at Mayo Clinic, Rochester, Minnesota, between 1976 and 2004. RESULTS: Urticarial papules were the most frequent clinical presentation followed by blisters and rash. Pruritus was the cardinal symptom. Lesions presented initially on the legs, thighs, back, and chest. Direct immunofluorescence had the highest diagnostic test sensitivity. Systemic corticosteroids were the mainstay of treatment. Fetal and maternal outcome was good in all cases. LIMITATIONS: This was a retrospective, single-institution study. CONCLUSIONS: This condition can be easily confused with other dermatoses of pregnancy, for example, pruritic urticarial papules of pregnancy. Biopsy for direct immunofluorescence is the preferred test for confirmation of diagnosis. On the basis of good patient outcomes, conservative treatment seems warranted.

Adolescent↗
PubMed1990

Western blot analysis of the antigen in pemphigoid gestationis.

Using an immunoblotting technique, sera from 25 patients with pemphigoid gestationis were examined and tested against epidermal and dermal extracts of normal skin. The major antigen recognized by seven patients' sera was a molecule of 180 kDa, pemphigoid gestationis antigen, extractable only from the epidermis. Sera from 18 patients with bullous pemphigoid were studied as positive controls and the major antigen recognized was a larger molecule of 220 kDa. There was some degree of shared recognition of antigens with three patients with pemphigoid gestationis recognizing the 220 kDa bullous pemphigoid antigen. In addition one bullous pemphigoid serum recognized the 180 kDa pemphigoid gestationis antigen. The dominant pemphigoid gestationis antigen, however, differs from bullous pemphigoid antigen.

Autoantigens↗
PubMed2003

[Pemphigoid gestationis: a study of 15 cases].

INTRODUCTION: Pemphigoid gestationis is a rare subepidermal bullous dermatosis generally occurring during the 2(nd) or 3(rd) quarter of gestation or in the postpartum period in women who already have been pregnant. OBJECTIVE: The aim of this work is to draw a profile of the epidemiology, clinical aspects, treatment and evolution of the disease by studying hospital series. METHODS: In this retrospective study, 15 cases of pemphigoid gestationis confirmed by direct immunofluorescence, followed in the department of dermatology between 1983 and 1999, were included. RESULTS: The age of onset was 19 to 39 years (mean age: 30 years). In 73% of cases, pemphigoid gestationis occurred in women who had already been pregnant, and appeared during the last 3 months of gestation in 11 patients. In all cases, purities was the first symptom, followed by a erythematous maculopapular eruption. In the steady state of the disease, all patients had annular confluent erythematous papules with herpes, like vesicles predominant in the umbilicus. The diagnosis of pemphigoid gestationis was confirmed by direct immunofluorescence in all cases demonstrated linear staining of C3 at the basement membrane zone. Systemic corticosteroids (0.5-1mg/kg/day) were used in 54% of reported cases. Dapsone was efficient in 26% of patients. 20% of patients were treated with oral antihistamine and topical glucocorticoid. Recurrence occurred in postpartum in 53,3% of patients. Two patients had recurrence during the following pregnancies. CONCLUSION: PG remains a rare dermatitis of pregnancy. Our series is comparable to the literature: the late occurrence of PG during the course of pregnancy, the high frequency of multigravida women, the lack involvement in the newborn, however with some particularities: the frequent involvement of the face and the efficiency of dapsone.

Adrenal Cortex Hormones↗