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Bullous Pemphigoid (bullou + pemphigoid)
Selected AbstractsHemidesmosome protein dynamics in live epithelial cellsCYTOSKELETON, Issue 2 2003Daisuke Tsuruta Abstract Hemidesmosomes mediate stable anchorage of epithelial cells to laminin-5 in the basement membrane zone and have been likened to spot-welds. Indeed, it has been assumed that hemidesmosomes are not dynamic, at least when compared to other matrix adhesion sites including focal contacts. We tested this notion by monitoring the fate of green fluorescent protein (GFP)-tagged human integrin ,4 subunit (GFP-h,4) and GFP-tagged 180-kD human bullous pemphigoid (BP) autoantigen (GFP-BP180) in live cultures of 804G cells that assemble numerous mature hemidesmosomes. In subconfluent 804G cells, both GFP-h,4 and GFP-BP180 protein clusters are not stable but assemble into and disassemble out of cat paw,like arrays at a relatively rapid rate. In confluent populations of 804G cells, although some cat paw,like clusters of both GFP-h,4 and GFP-BP180 are stable over periods of >60 min, other GFP-h,4 and GFP-BP180 protein arrays form and/or disappear during the same time period. Moreover, individual labeled particles show considerable motility in the plane of the membrane. Fluorescence recovery after photobleaching analyses provide a further indication of the dynamics of hemidesmosome proteins. In particular, bleached GFP-h,4 protein clusters in confluent cells recover signal within about 30 min, indicating that there is a relatively rapid turnover of hemidesmosome components in protein arrays clustered along the substratum attached surface of a cell. The rate of recovery is dependent on an intact microfilament system. In sharp contrast, bleached GFP-BP180 protein clusters in confluent cells fail to recover signal even when observed for longer than 60 min. To evaluate hemidesmosome protein dynamics in motile cells, we monitored GFP-h,4 and GFP-BP180 in 804G cells populating scrape wound sites in vitro. In these migratory cells, which lack mature hemidesmosomes, integrin ,4 subunit and BP180 protein clusters progressively assemble and disassemble into linear and cat-paw arrays. In summary, hemidesmosome protein clusters, like their counterparts in focal contacts, are dynamic. We discuss these results in relation to hemidesmosome functions. Cell Motil. Cytoskeleton 54:122,134, 2003. © 2003 Wiley-Liss, Inc. [source] Novel ELISA systems for antibodies to desmoglein 1 and 3: correlation of disease activity with serum autoantibody levels in individual pemphigus patientsEXPERIMENTAL DERMATOLOGY, Issue 5 2010Enno Schmidt Please cite this paper as: Novel ELISA systems for antibodies to desmoglein 1 and 3: correlation of disease activity with serum autoantibody levels in individual pemphigus patients. Experimental Dermatology 2010; 19: 458,463. Abstract:, Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are intraepidermal blistering skin diseases. PV is characterised by autoantibodies directed against desmoglein (Dsg) 3 and in patients with the mucocutaneous variant also against Dsg 1, whereas in PF, only Dsg 1 is targeted. Here, ectodomains of Dsg 3 and Dsg 1 were recombinantly expressed in a human cell line (HEK293) and applied as authentic solid phases in ELISA test systems. Autoantibodies against Dsg 3 and/or Dsg 1 could be detected in 71 (100%) of 71 PV sera and against Dsg 1 in 48 (96%) of 50 PF sera. Control sera showed reactivity with Dsg 3 and Dsg 1 in 0.2% and 0.7%, respectively, of 401 healthy blood donors and in 2.1% of 48 randomly selected patients with bullous pemphigoid. No reactivity with Dsg 1 and 3 was detected in 21 patients with linear IgA disease. For both pemphigus variants, a statistically significant correlation between clinical severity and autoantibody levels was observed as demonstrated for 10 PV and 5 PF patients. In conclusion, the use of the ectodomains of Dsg 3 and 1 as target antigens expressed in a human cell line resulted in sensitive and specific ELISA systems for both diagnosis and monitoring of PV and PF. [source] Mast cells: novel clinical perspectives from recent insightsEXPERIMENTAL DERMATOLOGY, Issue 5 2009Manfred Kneilling Abstract:, Mast cells are still generally viewed as mediators of type I allergic or pseudoallergic reactions. Research over the past 10 years revealed that our view was too small and that mast cells are of key importance in innate immunity and also types II, III and IV adaptive immune reactions. Understanding their role in modulating and amplifying of inflammatory responses provides important insights into the pathogenesis of skin diseases such as psoriasis, atopic dermatitis, bullous pemphigoid or the control of infections. This helps us to understand the course of these diseases, their trigger mechanisms, and, the new role of agents, which can modulate the function of mast cells. These insights will help to develop new therapeutic approaches. [source] Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseasesEXPERIMENTAL DERMATOLOGY, Issue 2 2007Takashi Matsushita Abstract:, Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma. [source] Frequency, function and CLA expression of CD4+CD25+FOXP3+ regulatory T cells in bullous pemphigoidEXPERIMENTAL DERMATOLOGY, Issue 1 2007Anne Rensing-Ehl Abstract:, Bullous pemphigoid (BP) is an autoimmune blistering skin disease associated with autoantibodies to collagen XVII and tissue-separation along the dermo-epidermal junction. We addressed the question whether the loss of tolerance in BP patients is associated with a reduction and/or functional impairment of CD4+CD25+FOXP3+ regulatory T cells, which are essential for the active maintenance of self tolerance. The relative and absolute frequency of CD4+CD25+ and CD4+CD25high regulatory T cells in the peripheral blood of newly diagnosed, untreated patients was similar to that of healthy controls. Interestingly, more than 50% of circulating CD4+CD25high regulatory T cells from both patients as well as healthy controls expressed cutaneous lymphocyte-associated antigen. Considerable numbers of FOXP3+ cells were detected in lesional skin of patients. CD4+CD25+ regulatory T cells of patients were functionally intact as assessed by their ability to suppress allogeneic as well as antigen-specific T-cell proliferation. These data argue against a general defect of CD4+CD25+FOXP3+ regulatory T cells in patients with BP. [source] Genetic variation in COL17A1 and the development of bullous pemphigoidEXPERIMENTAL DERMATOLOGY, Issue 3 2004Samantha Winsey Background: Bullous pemphigoid (BP) is an autoimmune blistering disease of the skin characterized by autoantibody attack on collagen XVII. Objectives: To characterize the genetic complexity of COL17A1, the gene which encodes for the autoantigen collagen XVII. The data will be used to determine whether there is an association between polymorphisms and haplotypes of COL17A1 and genetic susceptibility to development of BP. Methods: The genetic complexity in COL17A1 was deduced by screening and then sequencing the gene. Haplotypes were constructed from the resulting polymorphisms using the statistical programme PHASE. The linkage disequilibrium (D,) between the polymorphisms was deduced from haplotypic data using the statistical programme GOLD. Association of the polymorphisms and haplotypes was tested for, in a cohort of BP patients and controls. Results: Screening of COL17A1 for genetic variation was carried out in 29 individuals of North European caucasoid origin, and it revealed 19 single-nucleotide polymorphisms in approximately 14.7 kb of sequence. These variants resulted in 60 different haplotypes in 191 individuals, of which 13 occurred above 1% in the population. D, between the variants was found to be extensive, have a low correlation with physical distance and to extend over 33.8 kb. No association was found with any of the polymorphisms or haplotypes and development of BP, when tested for, in a cohort of patients and controls. Conclusion: This study provides an extensive description of the genetic variation in COL17A1 and shows no association of the genetic variants with susceptibility to BP. [source] Eosinophilic spongiosis in a case of lichen sclerosus: a marker for superimposed bullous pemphigoid?HISTOPATHOLOGY, Issue 7 2008N Leonard No abstract is available for this article. [source] An itchy vesiculobullous eruption in a patient with chronic lymphocytic leukaemiaINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 12 2004B. Cocuroccia Summary Exaggerated reactions to insect bites are characteristic of patients with haemoproliferative disorders, particularly chronic lymphocytic leukaemia (CLL). Skin lesions usually appear after the diagnosis of leukaemia and seem unrelated to laboratory findings, disease course or therapy. Rarely, the eruption may precede the diagnosis of the haematologic malignancy. The patients usually do not recall of insect bites, and the diagnosis may require histological and laboratory investigations to exclude specific lesions or autoimmune bullous diseases. Lesions may run a chronic course and represent a therapeutic challenge. Here, we report an adult patient with CLL who developed itchy recurrent papulovesicular and bullous lesions. Differential diagnosis was made with cutaneous specific lesions of CLL, bullous pemphigoid and pemphigus vulgaris, but laboratory and histological investigations confirmed the diagnosis of an insect bite reaction. The patient was treated with oral H1 anti-histamines and topical corticosteroids under occlusion, with marked improvement after 10 days. [source] Childhood bullous pemphigoid: a case report and 10-year follow upINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 5 2007Ana María Sáenz MD, Assist. Bullous pemphigoid (BP) is a very rare acquired immunbullous disease in children and infants. We report the case of a 15-month-old boy referred to our service with multiple tense bullae located predominantly on the trunk, neck and proximal portion of the limbs. Palms, soles and oral mucosa were also affected. Histopathologic and immunopathologic features were characterized with bullous pemphigoid. The patient responded well to systemic steroids, with improvement of his condition. After a 10-year follow up the patient was no longer suffering from the disease. [source] Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, GermanyJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 5 2009Franziska Bertram Summary Background: Only limited epidemiologic data are available on autoimmune bullous diseases. Improved diagnostic tools should have led to an increased incidence. To test this hypothesis, all patients with autoimmune bullous disorders who were treated in the Department of Dermatology at the University of Würzburg, Germany, between January 2001 and June 2002 were analysed prospectively. Patients and Methods: Epidemiologic data of patients diagnosed with an autoimmune bullous disease during this time period were registered and statistically evaluated. Diagnosis was based on the clinical picture and specific immunopathological findings. Only patients from Lower Franconia, a well-defined administrative region of Southern Germany, were included into this study. Results: During the study period, 41 patients with an autoimmune bullous disease were diagnosed, including 27 with bullous pemphigoid, 4 with pemphigoid gestationis and mucous membrane pemphigoid, 2 with dermatitis herpetiformis and linear IgA disease, and 1 with epidermolysis bullosa acquisita and pemphigus vulgaris, respectively. The highest incidence was calculated for bullous pemphigoid (13.4 per 1 million inhabitants per year) followed by pemphigoid gestationis (2.0) and mucous membrane pemphigoid (2.0). Patients with mucous membrane pemphigoid were found to have the highest mean age at disease onset (76 years) followed by patients with bullous pemphigoid (74 years). Conclusions: This is the first prospective study on the incidence of autoimmune bullous disorders. Subepidermal blistering autoimmune diseases were shown to be more frequent than previously reported for Central Europe. This is most likely due to improved diagnostic tools for and increased awareness of these diseases. [source] Pemphigoid vegetans: a case report and review of the literatureJOURNAL OF CUTANEOUS PATHOLOGY, Issue 12 2008Jinah Kim Pemphigoid vegetans is an exceptionally rare intertriginous variant of bullous pemphigoid characterized by vegetative and purulent lesions present in the groin, axillae, thighs, hands, eyelids and perioral regions. The clinical, histopathological and immunofluorescent profile of a new case of pemphigoid vegetans in a 79-year-old man is reported. Our patient had papillomatous plaques with pustules in the bilateral inguinal folds, which clinically resembled pemphigus vegetans. Also suggesting pemphigus vegetans, an initial skin biopsy showed eosinophilic spongiosis, while a second biopsy showed histological and immunological features diagnostic of pemphigoid. Because only a few cases of pemphigoid vegetans have been reported in the literature, clinical and morphological data are scant. Most reported cases were successfully treated with topical antibiotics or steroids; therefore, appropriate diagnosis of this rare lesion will assist management. [source] Histopathologic Differential Diagnosis of Subepidermal Cutaneous Blisters and Erosions at AutopsyJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005C. Kovarik The histopathologic differential diagnosis of subepidermal blisters is broad and most commonly includes primary dermatologic diseases (i.e. bullous pemphigoid), secondary blistering conditions (i.e. bullous lichen planus), and drug reactions (i.e. toxic epidermal necrolysis); however, when examining blister specimens taken during autopsy, several other entities need to be added to the differential diagnosis. For one year, we biopsied cutaneous blisters and erosions found during autopsies performed at the Dallas County Medical Examiner's Office and examined them histologically. The objective of this study was to determine the primary cause of blisters and erosions seen at autopsy, characterize them histologically in order to allow differentiation from other blistering conditions, and emphasize causes of subepidermal blisters that are often not considered in the differential diagnosis. We present eight representative cases in order to illustrate the following points. The majority of blisters and erosions examined were subepidermal and secondary to physical causes, such as burns, defibrillation, submersion, fetal maceration, and decomposition. Although primary skin conditions need to be considered when examining blisters and erosions at autopsy, the most common entities are secondary to physical causes, and these need to be included in the differential diagnosis in order to obtain an accurate diagnosis. [source] Bullous pemphigoid detection by micro-Raman spectroscopy and cluster analysis: structure alterations of proteinsJOURNAL OF RAMAN SPECTROSCOPY, Issue 11 2005Erez Azrad Abstract In this study, micro-Raman spectroscopy was used, for the first time, to detect spectral changes between healthy and diseased skin tissues with bullous pemphigoid (BP). The spectral changes provide information about the biochemical alterations between normal skin and blistered and nonblistered regions in samples diagnosed histopathologically as BP. Raman spectra, characterized by many peaks, revealed the molecular composition of the different skin layers, stratum corneum, epidermis and dermis of normal skin. Comparison of spectra monitored at the dermoepidermal junction (DEJ) of healthy skin with those of blisters caused by BP showed evidence for large variations in the amide I and III regions. The alterations of the protein content, amide I and III, are a result of the appearance of immunoglobulin G (IgG) and fibrin, characteristics of BP. Indication for the disease at early stages was obtained from changes in protein content, evidenced in the measured spectra assisted by cluster analysis. The method employed here can contribute to the nearly real-time diagnosis and to a better understanding of the physical and biomolecular processes effected by BP, and might have implications on other skin diseases. Copyright © 2005 John Wiley & Sons, Ltd. [source] Basement membrane changes in lichen planopilarisJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 11 2009K Al-Refu Abstract Background, Lichen planopilaris (LPP) is an inflammatory disease that affects the scalp and tends to produce cicatricial alopecia. The inflammatory process frequently results in the disruption of the basal cell of the external root sheath of the hair follicle. Objectives, To investigate the alterations in the basement membrane zone (BMZ) in LPP by immunohistochemistry. Methods, Skin biopsies from six patients with LPP plus six normal controls were studied by immunohistochemistry with antibodies to the following BMZ components: cytokeratin 5, cytokeratin 14, BP230 (bullous pemphigoid), BP180, plectin, laminin 5, collagen IV and collagen VII. Results, The localization and staining of the hemidesmosome, laminin and collagen components were strikingly different in the inflamed follicular epithelium when compared to the uninvolved follicles or interfollicular epithelium in active LPP lesions. The hemidesmosome-associated complexes were weakly expressed and discontinuous in involved hair follicles. The expression of laminin-5, type IV collagen and type VII collagen was disrupted and not linear along the BMZ with finger-like projections of the staining protruding into the dermis. The expression of the intermediate filaments was normal. Conclusion, These alterations in the BMZ in LPP may explain the abnormal healing at follicular level which leads to irreversible hair loss and scarring in this condition. [source] A case of bullous pemphigoid clinically mimicking subcorneal pustular dermatosisJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 5 2009T Dereli [source] Treatment of bullous pemphigoid with enteric-coated mycophenolate sodiumJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 4 2007U Tursen [source] Topical class I corticosteroids in 10 patients with bullous pemphigoid: correlation of the outcome with the severity degree of the disease and review of the literatureJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 2 2004A Stockman ABSTRACT Background, Treatment of bullous pemphigoid (BP) with systemic immunosuppressive agents, in particular with systemic corticosteroids, has many long-term side-effects. A dozen reports were published regarding the efficacy of topical corticosteroids in the treatment of bullous pemphigoid. Objective, To evaluate the efficacy of potent class I topical corticosteroids in relation to the affected body surface area (BSA) in patients with bullous pemphigoid and to review the literature. Methods, An open prospective trial with 10 patients with BP with measurement of the affected BSA. Treatment protocol consisted of three steps: potent class I topical corticosteroid treatment, systemic tetracyclines and systemic corticosteroids. Follow-up period was between 24 and 72 months. Results, Our study suggests a correlation between the success rate of topical corticosteroid treatment and the body surface area initially affected: all patients with an affected BSA of less than 20% healed with topical treatment only. The patients with more than 40% affected BSA needed systemic treatment with steroids. Conclusion, Topical class I corticosteroids seem to be effective in healing lesions of BP, especially if less than 20% of the BSA is affected. This study comprises only 10 patients, making further studies necessary to draw definite conclusions. [source] Treatment of bullous pemphigoid: what's new?JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2003U Wollina [source] Bullous pemphigoid in Liguria: A 2-year surveyJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 4 2001E Cozzani Abstract Background The epidemiology of bullous pemphigoid (BP) is not clear because of the heterogeneity of the disease, and its possible association with internal malignancies has been under debate for many years. We report the findings of a 2-year study on incident BP cases in the Liguria region of Italy. Subjects and methods Thirty-two patients with BP were collected over the 2-year period. Diagnosis was made based on clinical findings and confirmed by histology, direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) with salt-split skin and monkey oesophagus, and immunoblotting (IB). All patients were thoroughly investigated for possible malignancies and all were followed up for 6 months to monitor the response to treatment. Results DIF showed linear deposits at the dermoepidermal junction in all but one patient. IIF gave positive findings for 15 sera tested with monkey oesophagus and 20 tested with salt-split skin. IB gave positive findings in 19 cases. There was a malignancy in six cases, but no clinical or immunological features that could be considered to predict this occurrence. Conclusions The findings of this study are in accordance with most of the data found in the literature, including the fact that IgG serum levels did not predict the course of the disease. Contrary to previous indications, IgE levels were not indicative of disease course either. Mucosal lesions, erythema multiform-like lesions, negative IIF findings and antibodies to AgPB2 were not a prediction for the development of malignancy. [source] PL1 Subepithelial bullous diseases , topic overviewORAL DISEASES, Issue 2006M Mravak-Stipeti Subepithelial bullous diseases comprise the group of mucocutaneous autoimmune blistering diseases characterized by subepithelial separation and the deposition of immunoglobulin and complement against several antigens along the basement membrane zone (BMZ). This result in spectrum of diseases that affect skin, oral mucosa, and other mucosal membranes and include bullous pemphigoid (BP), mucous membrane (cicatricial) pemphigoid (MMP), linear IgA disease (LAD), and chronic bullous dermatosis of childhood (CBDC). The most common clinical features are oral erosions, desquamative gingivitis and conjunctival fibrosis, as well as skin lesions, predominantly in older female population. The heterogeneity of clinical presentation and diversity of target autoantigens have contributed to difficulties in characterizing this condition immunologically. In addition to the clinical presentation and a subepithelial vesicle or bullae on routine histologic analysis, the diagnosis is based on direct and indirect immunofluorescence studies. The nature of the disease is determined by the target antigens in the epithelium and BMZ such as antigen 180 (BP180), antigen 230 (BP230), laminin 5, and beta 4 integrin. Circulating IgG and IgA antibodies bind to different epitopes of BP180. The use of salt-split skin substrate enables differentiation between epidermal and dermal 'binders'. Since the antigen and the antibody titer appear to have direct relationships with the disease severity, and a combination of clinical finding and antibody titer provides valuable prognostic data, these investigations should be carried out routinely. Clinicians should recognize clinical spectrum of SBD, the histopathologic and immunopathologic characteristics, the differential diagnosis, the treatment, and the natural history of the disease. Involvement of oral medicine specialists, dermatologists, ophthalmologists, otolaryngologists and gastroenterologists contribute to early diagnosis and will aid in providing SBD patients with the highest quality of care. [source] Bullous Pemphigoid in Late Childhood Successfully Treated with Mycophenolate Mofetil as an Adjuvant TherapyPEDIATRIC DERMATOLOGY, Issue 5 2010JOHN C. FOX M.D. We report a 16-year-old girl with bullous pemphigoid who achieved disease remission with mycophenolate mofetil as an adjuvant therapy. [source] A Case of Juvenile Bullous Pemphigoid, Successful Treatment with Diaminodiphenylsulfone and PrednisonePEDIATRIC DERMATOLOGY, Issue 1 2009Karen A. Marcus M.D. Because of the wide variety of bullous disorders and the considerable clinical overlap between them, it is difficult to differentiate one from the other on clinical features alone. Appropriate additional investigations are required to confirm the diagnosis. These include routine histologic examination of the skin, in addition to immunohistochemical staining and immune serology. Here, we present a rare case of juvenile bullous pemphigoid, which we will use to illustrate the difficulties encountered in the diagnostic process and to show how acquired blistering disorders of childhood should be approached. [source] Childhood Bullous Pemphigoid: Report of a Case with Life-Threatening Course During Homeopathy TreatmentPEDIATRIC DERMATOLOGY, Issue 2 2004Stéphane Kuenzli M.D. We describe a 9-month-old child who developed bullous pemphigoid while she was being treated for presumptive atopic eczema with a homeopathic regimen comprising sulfur, mercury, cantharides, and Rhus (Toxicodendron). She had generalized bullae and a progressive worsening of her general condition with asthenia, dehydration, malnutrition. While the role of homeopathy in triggering the disease remains unclear, our observation attests to the potential life-threatening course of childhood BP in instances where appropriate treatment is withheld. [source] Vulvar Cicatricial Pemphigoid of ChildhoodPEDIATRIC DERMATOLOGY, Issue 1 2004A. Schoeffler M.D. A diagnosis of localized bullous pemphigoid or cicatricial pemphigoid was made on the basis of immunohistologic data. Since the lesions were unresponsive to topical corticosteroids but healed completely on dapsone at a dosage of 1.5 mg/kg/day, we favor the diagnosis of vulvar cicatricial pemphigoid. Only two such cases have been reported thus far. The diagnostic criteria and therapeutic modalities are discussed. [source] Vulvar Disease in Children: A Clinical Audit of 130 CasesPEDIATRIC DERMATOLOGY, Issue 1 2000Gayle Fischer M.D. Of the patients, 41 (33%) had atopic or irritant dermatitis, 23 (18%) had lichen sclerosus, 21 (17%) had psoriasis, 15 (12%) had vulvar lesions, most often hemangiomas and nevi, and 13 (10%) had streptococcal vulvovaginitis. Diagnoses less frequently seen were staphylococcal folliculitis (four patients), labial fusion (three patients), genital warts (two patients), molluscum contagiosum of the vulva only (one patient), vulvar bullous pemphigoid (two patients), scabies nodules (one patient), erythema annulare centrifugum (one patient), tinea (two patients), and vitiligo (one patient). We also encountered vulvar presentations of systemic diseases (varicella, staphylococcal scalded skin syndrome, and Henoch,Schönlein purpura, all one patient each). We did not see candidal vulvovaginitis in this age group nor did we encounter bacterial infection with pathogens other than Staphylococcus aureus and S. pyogenes. [source] Tetracycline and niacinamide control bullous pemphigoid but not pemphigus foliaceus when these conditions coexistTHE JOURNAL OF DERMATOLOGY, Issue 7 2010Junko SHIOHARA Abstract Pemphigus and pemphigoid are different types of autoimmune bullous disease and can occur in the same patient. We report a female patient with this condition. At first, we diagnosed her with bullous pemphigoid, and we treated her with tetracycline, niacinamide and a topical steroid. Tense bullas disappeared shortly after that, but crusted erythemas mainly on her head and trunk persisted. We examined BP180 and desmoglein 1 enzyme-linked immunosorbent assays, and also histological features, which showed coexistence of bullous pemphigoid and pemphigus foliaceus concurrently. Therefore, we tried prednisolone, which could control both conditions. This case showed that tetracycline and niacinamide could control bullous pemphigoid, but could not control pemphigus foliaceus, and that prednisolone was effective for both conditions. [source] What's new in bullous pemphigoidTHE JOURNAL OF DERMATOLOGY, Issue 3 2010Hideyuki UJIIE Abstract Bullous pemphigoid (BP) is the most common autoimmune blistering disease. BP patients have autoantibodies against type XVII collagen (COL17, also called BP180 or BPAG2), a type II transmembrane protein that spans the lamina lucida and projects into the lamina densa of the epidermal basement membrane. The non-collagenous 16A domain of COL17 is considered to contain pathogenic epitopes of BP. The transfer of immunoglobulin (Ig)G from BP patients fails to cause blisters on mouse skin probably due to differences between humans and mice in the amino acid sequence of NC16A pathogenic epitope of COL17. Passive transfer of rabbit IgG antibodies against the murine homolog of human COL17 NC16A triggers immune reactions to COL17 in mice, including complement activation, mast cell degranulation and neutrophilic infiltration, resulting in dermal,epidermal separation. Recent studies using COL17-humanized mice that express human COL17 but lack murine COL17 were the first to demonstrate the pathogenicity of anti-COL17 human BP IgG autoantibodies in vivo. These new findings provide a greater understanding of BP pathomechanisms and facilitate the development of novel specific and efficient therapeutic strategies for BP. [source] Immunoglobulin A antibodies against desmoglein 1, envoplakin, periplakin and BP230 in a patient with atypical bullous pemphigoidTHE JOURNAL OF DERMATOLOGY, Issue 3 2010Fumi YAMAKI Abstract Bullous pemphigoid is an autoimmune subepidermal blistering disease associated with autoantibodies against BP180 and BP230. We report herein a rare case of bullous pemphigoid with newly formed annular erythematous lesions when bullous skin lesions were in remission. Various immunological studies revealed immunoglobulin (Ig)A antibodies against desmoglein 1, envoplakin, periplakin and BP230 in addition to IgG antibodies against BP180 and BP230. These clinical and immunological changes in a patient are a rare event, suggesting an epitope-spreading phenomenon. [source] Paraneoplastic bullous pemphigoid: Clinical disease activity correlated with enzyme-linked immunosorbent assay index for the NC16A domain of BP180THE JOURNAL OF DERMATOLOGY, Issue 1 2009Hee-Jin SONG No abstract is available for this article. [source] Efficacy of interferon-, in patients with refractory bullous pemphigoidTHE JOURNAL OF DERMATOLOGY, Issue 11 2007Takekuni NAKAMA ABSTRACT Bullous pemphigoid (BP) usually responds well to conventional therapies, although some severe cases show less response to various therapies or develop side-effects due to long-term drug administration using high doses. Most BP patients are elderly and are thus prone to systemic deterioration or unfavorable outcome. In the present study, we investigated the efficacy of interferon-, on severe BP patients resistant to conventional therapies. Interferon-, was administered to 10 severe BP patients at a dose of 2 million Japan reference units (JRU) once a day for 7 consecutive days by i.v. infusion in addition to oral corticosteroids. The degree of improvement in the clinical symptoms, serum interleukin (IL)-4, IL-5, and plasma RANTES concentrations, as well as the results of indirect immunofluorescence and BP180 enzyme-linked immunosorbent assay index values, were compared before and after the 7-day drug administration. Among the nine patients whose clinical symptoms were evaluated, an improvement was observed in all patients. Except for one patient, the serum IL-4 concentrations decreased and similar results were observed for the serum IL-5 concentration. All five patients in whom the plasma RANTES concentration was measured showed decreased levels. The indirect immunofluorescence titers decreased in only four patients. However, in seven patients in whom index values of BP180 enzyme-linked immunosorbent assay were evaluated, all patients showed significant decrease of the index values. These results suggest that, in severe BP patients refractory to conventional therapies, interferon-, in addition to oral corticosteroids is effective and thus should be considered for further clinical use. [source] | |||||||||||||