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Pemphigus
Kinds of Pemphigus Terms modified by Pemphigus Selected AbstractsMolecular identification and population dynamics of two species of Pemphigus (Homoptera: Pemphidae) on cabbageINSECT SCIENCE, Issue 2 2009Naiqi Chen Abstract The poplar petiole gall aphid, Pemphigus populitransversus Riley, has been one of the major pests on cruciferous vegetable in the Rio Grande Valley (LRGV) of Texas since the late 1940s. It normally migrates from poplar trees to cruciferous vegetables in the fall, and migrates back to the trees in early spring of the coming year. Some root-feeding aphids were found on cruciferous vegetables in late spring and early summer in 1998 and the following years. Those aphids have been identified as Pemphigus obesinymphae Moran. This discovery completely changed the current knowledge about the root-feeding aphids on cruciferous vegetables in the LRGV. Due to their small size, morphological and feeding similarities between P. populitransversus and P. obesinymphae, their identification and distinction are difficult. In this study, random amplification of polymorphic DNA (RAPD) and amplified fragment length polymorphism (AFLP) were used to distinguish these two species over a period of time when the two species occurred together, or separately, in cabbage fields. The two species occurred on cabbage at different times of the year, and overlapped from October to June. From May to October, both species migrated to their primary hosts. The apterous aphids found on cabbage in winter contained mainly P. obesinymphae, whereas in early spring more apterous P. populitransversus were recovered. The root-feeding aphids would feed on cabbage plants as long as this host was available even during the hot, dry summer in the LRGV, although their populations were generally low. Both RAPD and AFLP techniques were efficient in discriminating the two species that showed obviously genetic variability. These molecular techniques confirmed the existence of the two aphid species in apterous samples collected from the soil in cabbage fields in the LRGV, and the results performed by RAPD were confirmed by AFLP. Furthermore, the results suggest that RAPD technique was a better choice despite its reproducibility problem, as it was less time-consuming and required less technology, labor and expense than AFLP. [source] Pemphigus and diet: does a link exist?INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 3 2001Vincenzo Ruocco MD No abstract is available for this article. [source] Intravenous dexamethasone-cyclophosphamide pulse therapy in comparison with oral methylprednisolone-azathioprine therapy in patients with pemphigus: Results of a multicenter prospectively randomized studyJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 3 2005Intravenöse Dexamethason-Cyclophosphamid-Pulstherapie im Vergleich zu einer oralen Methylprednisolon-Azathioprin-Therapie bei Patienten mit Pemphigus-Erkrankungen: Ergebnisse einer multizentrischen, prospektiven, randomisierten Studie Azathioprin; Cyclophosphamid; Pemphigus; Pulstherapie Summary Background: Pemphigus is a potentially life-threatening autoimmune blistering skin disease usually treated with high-dose corticosteroids in combination with immunosuppressive drugs. In a multicenter, prospectively randomized study we compared efficacy and side effects of a dexamethasone-cyclophosphamide (D/C) pulse therapy with a methylprednisolone-azathioprine (M/A) therapy in 22,patients with newly diagnosed pemphigus vulgaris and pemphigus foliaceus. Patients and methods: The 11,patients of the M/A group were treated with daily doses of methylprednisolone (initially 2,mg/kg body weight) and azathioprine (2,,,2,5,mg/kg body weight) which were subsequently tapered. D/C pulse therapy in 11,patients consisted of intravenous administration of 100,mg dexamethasone/d on 3 consecutive days along with cyclophosphamide (500,mg) on day one. Pulses were initially repeated every 2,,,4 weeks and then at increasing intervals. In between the pulses, oral cyclophosphamide (50,mg) was given daily for 6,months. Results: Within 24,months after treatment initiation, 5/11,patients of the D/C group had a remission (complete remissions after discontinuation of therapy in 3,patients) and 6/11,patients had a progression. In the M/A group, there were remissions in 9/11,patients (complete remissions after discontinuation of therapy in 3,patients) and progression in 1/11,patients. There were more relapses in M/A therapy after remission than in D/C therapy. Side effects were more common in the M/A group. These differences were not significant (p > 0,05). Conclusion: Because of the high number of progressions in patients treated with D/C therapy, we can not confirm the encouraging results of earlier reports about pulse D/C therapy. Nevertheless D/C therapy seemed to be better tolerated and, in case of primary efficacy, was associated with fewer recurrences than M/A therapy. Zusammenfassung Hintergrund: Pemphiguserkrankungen sind potentiell lebensbedrohliche blasenbildende Autoimmunerkrankungen, die üblicherweise mit hochdosierten Kortikosteroiden in Kombination mit Immunsuppressiva behandelt werden. In einer multizentrischen, prospektiven, randomisierten Studie verglichen wir die Wirksamkeit und Nebenwirkungen einer Dexamethason-Cyclophosphamid (D/C)-Pulstherapie mit einer Methylprednisolon-Azathioprin (M/A)-Therapie bei 22,Patienten mit neu diagnostiziertem Pemphigus vulgaris und Pemphigus foliaceus. Patienten und Methoden: 11,Patienten der M/A-Gruppe wurden kontinuierlich oral mit Methylprednisolon (initial 2,mg/kg Körpergewicht/Tag) und Azathioprin (2,,,2,5,mg/kg Körpergewicht/Tag) behandelt; die Dosen wurden schrittweise reduziert. Die Therapie bei den 11,Patienten der D/C-Gruppe erfolgte durch intravenöse Gabe von 100,mg Dexamethason/Tag an 3 aufeinander folgenden Tagen und 500,mg Cyclophosphamid am ersten Tag. Die Pulstherapie wurde zunächst alle 2,,,4 Wochen, dann in längeren Abständen wiederholt. Im Intervall wurden 50,mg Cyclophosphamid/Tag oral für 6,Monate verabreicht. Ergebnisse: Innerhalb von 24,Monaten nach Therapiebeginn kam es bei 5 von 11,Patienten der D/C-Gruppe zu einer Remission (komplette Remission nach Absetzen der Therapie bei 3,Patienten); bei 6 der 11,Patienten verlief die Erkrankung progredient. In der M/A-Gruppe kam es bei 9 von 11,Patienten zu einer Remission (komplette Remission nach Absetzen der Therapie bei 3,Patienten) und bei einem Patienten zu einer Progression. In der M/A-Gruppe traten häufiger Rezidive nach Remission auf als in der D/C-Gruppe. Therapienebenwirkungen kamen in der M/A-Gruppe häufiger vor. Diese Unterschiede waren nicht signifikant (p > 0,05). Schlußfolgerungen: Aufgrund der hohen Anzahl von Progressionen bei Patienten der D/C-Gruppe können wir die positiven Ergebnisse früherer Berichte über die D/C-Pulstherapie nicht bestätigen. Dennoch scheint die D/C-Therapie, beim einzelnen Patienten einmal erfolgreich, seltener zu Rezidiven zu führen und möglicherweise auch besser verträglich zu sein als die M/A-Therapie. [source] Pemphigus and drug addictionJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 8 2009RV Puca [source] Pemphigus vulgaris induced by radiotherapyJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 4 2004E Orion ABSTRACT Pemphigus can be triggered or induced by many insults, such as drugs, infections, viruses and neoplasms. X-ray-induced pemphigus has been recorded only rarely in the literature. We describe the case of a woman with pemphigus vulgaris in remission, who relapsed 1 week after completion of an irradiation course for breast cancer, in the exact location of the radiation. We review the previous 15 cases from the literature and outline the common features of those cases. [source] Number II Pemphigus vulgarisORAL DISEASES, Issue 3 2005M Black Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering. Pemphigus vulgaris (PV), the most common variant, is characterized by circulating IgG antibodies directed against desmoglein 3 (Dsg3), with about half the patients also having Dsg1 autoantibodies. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles and ethnic groups such as Ashkenazi Jews and those of Mediterranean and Indian origin, are especially liable. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examination are essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected using both normal human skin and monkey oesophagus or by enzyme-linked immunosorbent assay. Before the introduction of corticosteroids, PV was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using corticosteroids, with azathioprine or other adjuvants or alternatives but newer therapies with potentially fewer adverse effects, also appear promising. [source] Paraneoplastic Pemphigus with Bronchiolitis Obliterans in a ChildPEDIATRIC DERMATOLOGY, Issue 3 2003M.D., Winnie A. Mar Most cases have been reported in adults and the number of childhood cases in the current literature is limited. We describe a young patient with PNP who was initially misdiagnosed as having recurrent Stevens,Johnson syndrome. This patient had an underlying inflammatory myofibroblastic tumor and subsequently developed fatal progressive bronchiolitis obliterans. [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] An evaluation of the usefulness of mycophenolate mofetil in pemphigusBRITISH JOURNAL OF DERMATOLOGY, Issue 1 2003A.M. Powell SummaryBackground Pemphigus is a group of autoimmune blistering diseases of the skin and/or mucous membranes requiring management with immunosuppressive therapy. The optimal therapeutic regimen would rapidly induce remission and maintain effectiveness with minimal adverse effects in the long term. Objectives The present study describes our experience of the addition of mycophenolate mofetil (MMF) to prednisolone in the management of severe, refractory pemphigus. Methods Patients with active, refractory pemphigus were treated with MMF. Our series included 12 cases of pemphigus vulgaris, four cases of pemphigus foliaceous and one case of paraneoplastic pemphigus. All patients were monitored to assess disease control and mycophenolate toxicity. Results Of the 17 cases, MMF has been of benefit to 12. MMF was well tolerated and there were no treatment withdrawals because of safety concerns. Conclusions We found that MMF permitted a reduction in prednisolone dosage without disease relapse. [source] Pemphigus following tetanus and diphtheria vaccinationBRITISH JOURNAL OF DERMATOLOGY, Issue 1 2002E. Cozzani No abstract is available for this article. [source] Pemphigus Foliaceus Masquerading as Postoperative Wound Infection: Report of a Case and Review of the Koebner and Related Phenomenon following Surgical ProceduresDERMATOLOGIC SURGERY, Issue 2 2005Adam M. Rotunda MD Background The Koebner phenomenon, also known as the isomorphic response, is the development of preexisting skin disease following trauma to uninvolved skin. Various cutaneous disorders have been described to arise at surgical wounds and scars. Moreover, dermatologic procedures, such as cold-steel and laser surgery, can evoke koebnerization. Objective To describe a case of pemphigus foliaceus arising in postoperative wounds and to present a review of dermatologic disorders triggered by surgical procedures. Methods We report a case of pemphigus foliaceus initially presenting at sites of Mohs' micrographic surgery, shave biopsy, and cryotherapy and, subsequently, at a nonsurgical site. We reviewed the English literature in MEDLINE from November 1955 to April 2004 for reports of Koebner and related phenomenon following surgical procedures. Results To our knowledge, this is the first reported case of pemphigus foliaceus erupting at surgical and cryotherapy wounds. The clinical appearance can mimic wound infection. In addition to inducing preexisting disease, cutaneous procedures can also trigger the onset of new disease, which can either be limited only to the surgical site or subsequently become generalized. Conclusion Postoperative Koebner or related responses should be included in the differential diagnosis of poorly healing surgical wounds. Skin biopsies for histopathology and immunologic studies may be necessary for definitive diagnosis and optimal management. ADAM M. ROTUNDA, MD, ANAND R. BHUPATHY, DO, ROBERT DYE, MD, AND TERESA T. SORIANO, MD, HAVE INDICATED NO SIGNIFICANT INTEREST WITH COMMERCIAL SUPPORTERS. [source] Detection of pemphigus desmoglein 1 and desmoglein 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison with serum valuesEUROPEAN JOURNAL OF ORAL SCIENCES, Issue 5 2006Dimitrios Andreadis Although there is much literature on the detection of pemphigus and pemphigoid autoantibodies by enzyme-linked immunosorbent assay (ELISA) in serum, nothing is known about their presence in saliva. The aim of this study was to evaluate the salivary levels of these autoantibodies in pemphigus and pemphigoid patients. Autoantibodies against desmoglein3, desmoglein1, and BP180 were assayed, by ELISA, in serum and saliva samples of patients and healthy controls. The titres of autoantibodies against Dsg1/3 found in both serum and saliva of pemphigus patients showed a statistically significant correlation, suggesting that saliva may be a useful biological material for diagnostic purposes, in monitoring disease activity, as well as for the early detection of relapses. By contrast, the titres of autoantibodies against BP180 in the serum and saliva of bullous pemphigoid patients were not statistically related, and further study of the usefulness of the BP180 ELISA for saliva in this disease is needed. In addition, based on our results, the BP180 ELISA with a recombinant NC16a epitope failed to detect the autoantibodies against BP180 in the serum and saliva of mucous membrane pemphigoid patients. [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] Cholinergic control of epidermal cohesionEXPERIMENTAL DERMATOLOGY, Issue 4 2006Sergei A. Grando Abstract:, The non-neuronal cholinergic system of human epidermis includes the keratinocyte (KC) acetylcholine (ACh) axis composed of the enzymes mediating ACh synthesis and degradation, and two classes of ACh receptors, the nicotinic and muscarinic ACh receptors, mediating biological effects of the cutaneous cytotransmitter ACh. Regulation of KC cell,cell and cell,matrix adhesion is one of the important biological functions of cutaneous ACh. The downstream targets of ACh effects mediated by distinct ACh receptor subtypes include both the intercellular adhesion molecules, such as classical and desmosomal cadherins, and integrins mediating KC adhesion to a substrate. The signaling pathways include activation or inhibition of kinase cascades resulting in either up- or down-regulation of the expression of cell adhesion molecules or changes in their phosphorylation status, or both. The components of the KC ACh axis are involved in cutaneous blistering in patients with autoimmune pemphigus, junctional and dystrophic forms of epidermolysis bullosa, thermal burns, and mustard-induced vesication. Recent progress with the development of antiacantholytic therapies of patients with pemphigus using cholinomimetics indicates that cholinergic drugs may be a promising approach for other cutaneous blistering disorders. [source] Calcium and magnesium competitively influence the growth of a PMR1 deficient Saccharomyces cerevisiae strainFEMS MICROBIOLOGY LETTERS, Issue 2 2005Réka Szigeti Abstract PMR1, the Ca2+/Mn2+ ATPase of the secretory pathway in Saccharomyces cerevisiae was the first member of the secretory pathway Ca2+ ATPases (SPCA) to be characterized. In the past few years, pmr1, yeast have received more attention due to the recognition that the human homologue of this protein, hSPCA1 is defective in chronic benign pemphigus or Hailey,Hailey disease (HHD). Recent publications have described pmr1, S. cerevisiae as a useful model organism for studying the molecular pathology of HHD. Some observations indicated that the high Ca2+ sensitive phenotype of PMR1 defective yeast strains may be the most relevant in this respect. Here we show that the total cellular calcium response of a pmr1, S. cerevisiae upon extracellular Ca2+ challenge is decreased compared to the wild type strain similarly as observed in keratinocytes. Additionally, the novel magnesium sensitivity of PMR1 defective yeast is revealed, which appears to be a result of competition for uptake between Ca2+ and Mg2+ at the plasma membrane level. Our findings indicate that extracellular Ca2+ and Mg2+ competitively influence the intracellular Ca2+ homeostasis of S. cerevisiae. These observations may further our understanding of HHD. [source] Evaluation of coexistence of the Human Herpesvirus type 8 (HHV-8) infection and pemphigusINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 7 2010Naser Tayyebi Meibodi Associate Professor Background, Human Herpesvirus type 8 (HHV-8) is a new member of the herpes virus family, first found in the tissue of acquired immune deficiency syndrome (AIDS)-related Kaposi's sarcoma. Environmental factors including viral infection may play a role in the onset and/or development of pemphigus. Some studies based on polymerase chain reaction findings suggest an association between HHV-8 and pemphigus. The aim of this study is investigation of the association of pemphigus with HHV-8 and the relationship between inflammatory and acantholytic cells with HHV-8 infection. Methods, Tissue-extracted DNA from 41 paraffin-embedded skin tissues from patients first presenting with pemphigus was tested using nested PCR for the presence of HHV-8. A total of 37 cases had pemphigus vulgaris (PV) and 4 patients had pemphigus foliaceus (PF). For our control group, normal skin of 21 cosmetic surgery patients were included. Furthermore, microscopic slides with H&E staining were evaluated for the number of inflammatory and acantholytic cells per microscopic field. Results, Skin lesions from 13 of 37 patients (35.1%) with PV and 2 of 4 cases (50%) with PF were positive for HHV-8 DNA. All specimens in our control group were negative for this virus. Conclusion, HHV-8 infection might be a contributing factor in the initiation or development of pemphigus. [source] Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndromeINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 2 2009FNASC, FRAS(LOND.), Virendra N. Sehgal MD Paraneoplastic pemphigus is the term used for an exclusive subset of pemphigus. The clinical lesions may resemble pemphigus, pemphigoid, erythema multiforme, graft-vs.-host disease, or lichen planus. A common denominator in all patients is the concomitant occurrence of either occult or confirmed systemic neoplasm. It is imperative to confirm the diagnosis through microscopy, where intraepidermal suprabasal cleavage, epidermal acantholysis, dyskeratotic keratinocytes and vacuolar changes in the basal epidermis, interfacial dermatitis, and epidermal exocytosis can be seen. Furthermore, the deposition of immunoglobulin G (IgG) and complement in the epidermal intercellular spaces, detected by direct and/or indirect immunofluorescence, is equally crucial for confirming the diagnosis. [source] Paraneoplastic pemphigus in association with B-cell lymphocytic leukemia and hepatitis C: favorable response to intravenous immunoglobulins and prednisoloneINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 7 2007Mousumee Nanda MD No abstract is available for this article. [source] Rituximab: Applications in dermatologyINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2006Mitra M. Fatourechi Rituximab is a chimeric anti-CD20 monoclonal antibody which has been used extensively for B-lymphocytic malignancies. In addition, applications for autoimmune diseases have emerged in recent years. Case reports support the use of rituximab in certain dermatologic conditions, including paraneoplastic pemphigus, pemphigus vulgaris, graft versus host disease, and cutaneous B-cell malignancies. Clinical trials are lacking and would be an appropriate next step. [source] Chloroquine/hydroxychloroquine-induced pemphigusINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2006Gholamhosien Ghaffarpour MD No abstract is available for this article. [source] Intravenous dexamethasone-cyclophosphamide pulse therapy in comparison with oral methylprednisolone-azathioprine therapy in patients with pemphigus: Results of a multicenter prospectively randomized studyJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 3 2005Intravenöse Dexamethason-Cyclophosphamid-Pulstherapie im Vergleich zu einer oralen Methylprednisolon-Azathioprin-Therapie bei Patienten mit Pemphigus-Erkrankungen: Ergebnisse einer multizentrischen, prospektiven, randomisierten Studie Azathioprin; Cyclophosphamid; Pemphigus; Pulstherapie Summary Background: Pemphigus is a potentially life-threatening autoimmune blistering skin disease usually treated with high-dose corticosteroids in combination with immunosuppressive drugs. In a multicenter, prospectively randomized study we compared efficacy and side effects of a dexamethasone-cyclophosphamide (D/C) pulse therapy with a methylprednisolone-azathioprine (M/A) therapy in 22,patients with newly diagnosed pemphigus vulgaris and pemphigus foliaceus. Patients and methods: The 11,patients of the M/A group were treated with daily doses of methylprednisolone (initially 2,mg/kg body weight) and azathioprine (2,,,2,5,mg/kg body weight) which were subsequently tapered. D/C pulse therapy in 11,patients consisted of intravenous administration of 100,mg dexamethasone/d on 3 consecutive days along with cyclophosphamide (500,mg) on day one. Pulses were initially repeated every 2,,,4 weeks and then at increasing intervals. In between the pulses, oral cyclophosphamide (50,mg) was given daily for 6,months. Results: Within 24,months after treatment initiation, 5/11,patients of the D/C group had a remission (complete remissions after discontinuation of therapy in 3,patients) and 6/11,patients had a progression. In the M/A group, there were remissions in 9/11,patients (complete remissions after discontinuation of therapy in 3,patients) and progression in 1/11,patients. There were more relapses in M/A therapy after remission than in D/C therapy. Side effects were more common in the M/A group. These differences were not significant (p > 0,05). Conclusion: Because of the high number of progressions in patients treated with D/C therapy, we can not confirm the encouraging results of earlier reports about pulse D/C therapy. Nevertheless D/C therapy seemed to be better tolerated and, in case of primary efficacy, was associated with fewer recurrences than M/A therapy. Zusammenfassung Hintergrund: Pemphiguserkrankungen sind potentiell lebensbedrohliche blasenbildende Autoimmunerkrankungen, die üblicherweise mit hochdosierten Kortikosteroiden in Kombination mit Immunsuppressiva behandelt werden. In einer multizentrischen, prospektiven, randomisierten Studie verglichen wir die Wirksamkeit und Nebenwirkungen einer Dexamethason-Cyclophosphamid (D/C)-Pulstherapie mit einer Methylprednisolon-Azathioprin (M/A)-Therapie bei 22,Patienten mit neu diagnostiziertem Pemphigus vulgaris und Pemphigus foliaceus. Patienten und Methoden: 11,Patienten der M/A-Gruppe wurden kontinuierlich oral mit Methylprednisolon (initial 2,mg/kg Körpergewicht/Tag) und Azathioprin (2,,,2,5,mg/kg Körpergewicht/Tag) behandelt; die Dosen wurden schrittweise reduziert. Die Therapie bei den 11,Patienten der D/C-Gruppe erfolgte durch intravenöse Gabe von 100,mg Dexamethason/Tag an 3 aufeinander folgenden Tagen und 500,mg Cyclophosphamid am ersten Tag. Die Pulstherapie wurde zunächst alle 2,,,4 Wochen, dann in längeren Abständen wiederholt. Im Intervall wurden 50,mg Cyclophosphamid/Tag oral für 6,Monate verabreicht. Ergebnisse: Innerhalb von 24,Monaten nach Therapiebeginn kam es bei 5 von 11,Patienten der D/C-Gruppe zu einer Remission (komplette Remission nach Absetzen der Therapie bei 3,Patienten); bei 6 der 11,Patienten verlief die Erkrankung progredient. In der M/A-Gruppe kam es bei 9 von 11,Patienten zu einer Remission (komplette Remission nach Absetzen der Therapie bei 3,Patienten) und bei einem Patienten zu einer Progression. In der M/A-Gruppe traten häufiger Rezidive nach Remission auf als in der D/C-Gruppe. Therapienebenwirkungen kamen in der M/A-Gruppe häufiger vor. Diese Unterschiede waren nicht signifikant (p > 0,05). Schlußfolgerungen: Aufgrund der hohen Anzahl von Progressionen bei Patienten der D/C-Gruppe können wir die positiven Ergebnisse früherer Berichte über die D/C-Pulstherapie nicht bestätigen. Dennoch scheint die D/C-Therapie, beim einzelnen Patienten einmal erfolgreich, seltener zu Rezidiven zu führen und möglicherweise auch besser verträglich zu sein als die M/A-Therapie. [source] Smoking can be good for youJOURNAL OF COSMETIC DERMATOLOGY, Issue 2 2004R Wolf Summary Smoking is without doubt one of the greatest causes of avoidable illness and death in the modern world. Most well known is the relationship between smoking and numerous cancers, cerebrovascular and cardiovascular disease. Smoking and most especially nicotine, are, however, sometimes beneficial in certain diseases, including Parkinson's, Alzheimer's, allergic alveolitis, nausea and vomiting of pregnancy, pre-eclampsia, fibroids, carcinoma of body of uterus, ulcerative colitis, pyoderma gangrenosum, aphthous stomatitis and ulceration, pemphigus, herpes simplex and acne. In the immensely justifiable enthusiasm to discredit this dangerous activity, the mechanisms behind these beneficial effects tend to have been un-discussed or ignored. It is the aim of this paper to spur interest in the reasons for these effects. If the mechanisms are elucidated, therapeutic advances may be possible. [source] How does acantholysis occur in pemphigus vulgaris: a critical reviewJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2006Alessandro Lanza Background:, Pemphigus vulgaris is a life-threatening autoimmune blistering disease targeting skin and mucous membranes, characterized by disruption of keratinocytes' adhesion termed acantholysis. Today multiple classes of targets are considered to play a role in the genesis of the acantholysis; of these, the classical pemphigus antigens, desmosomal cadherins (desmoglein 1 and 3) are the best characterized and considered as the most important. Additional antigens include the novel epithelial acetylcholine receptors (,9 and pemphaxin). Thus, acantholysis in pemphigus seems to result from a cooperative action of antibodies to different keratinocyte self-antigens, but the mechanisms by which epithelial cleft occurs are not yet clearly understood. In fact, the binding of the autoantibodies to these targets generates a plethora of biological effects due, on one hand, to their direct interference with adhesive function and, on the other, to more complex events involving intracellular pathways that modify proteases activity or calcium metabolism, leading to loss of cell,cell adhesion. [source] Upregulation of P-cadherin expression in the lesional skin of pemphigus, Hailey-Hailey disease and Darier's diseaseJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2001Megumi Hakuno Background: Autoimmune blistering diseases, pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are known to be caused by binding of autoantibodies to the desmosomal cadherins, desmoglein 3 and desmoglein 1, respectively. Recently, mutations in the genes coding Ca2+ pumps leads to inherited blistering diseases, Hailey-Hailey disease (HHD) and Darier's disease (DD). Cadherins are a family of Ca2+ -dependent cell adhesion molecules and P-cadherin is one of the major cadherins expressed in the epidermis. Although detailed mechanisms of acantholysis of these blistering diseases have not been fully clarified, abnormal expression of cadherins caused by altered Ca2+ concentration due to the binding of autoantibodies to cell surface or by mutations in Ca2+ pumps is suggested to be involved in mechanisms of acantholysis of these atuoimmune and inherited blistering diseases. The purpose of the present study was to determine whether altered P-cadherin expression is present in these diseases. Method: Distribution patterns of P-cadherin in skin specimens from patients with PV (n=2), PF (n=2), HHD (n=4) and DD (n=3), were examined with confocal laser scanning microscopy using two anti-P-cadherin antibodies, 6A9 and NCC-CAD-299. Results: In normal control skin, P-cadherin expression was restricted to the basal layer. In contrast, positive immunostaining of P-cadherin was observed not only in the basal cells, but also in the suprabasal cells in lesional skin of all the acantholytic diseases. Conclusions: The present results clearly demonstrated that upregulation of P-cadherin expression occurs in the acantholysis in all the four blistering diseases PV, PF, HHD and DD. Upregulation of P-cadherin may be involved in the pathomechanism of both the autoimmune blistering diseases and the inherited blistering diseases. [source] A case of suspected contact dermatitis in a juvenile cynomolgus monkey (Macaca fascicularis)JOURNAL OF MEDICAL PRIMATOLOGY, Issue 2008Joanne Morris Abstract Background, A 2-year-old male cynomolgus monkey (Macaca fascicularis) presented with vesicular dermatitis exhibiting freshly ruptured blisters, crusts, and papules on the extremities and face. Methods, Complete blood count, serum chemistry, skin biopsy, skin scrape, and culture of a fresh blister were initially submitted for diagnostics. Results, Skin biopsy of the affected area revealed a non-suppurative eosinophilic dermatitis with mild thickening of the epidermis. Serum chemistry showed a marked eosinophilia (1.74 × 103/,l, 17.4%). All other results were within normal limits. Initial differentials included contact dermatitis, immune-mediated disease such as pemphigus or psoriasis. Repeated blood work and skin biopsies were collected as well as serum for allergen-specific IgE latex and food allergy testing. Herpes B virus was added to the differential list after an oral lesion was noted upon repeated physical examination and samples were collected for testing. Repeat blood work maintained a marked eosinophilia and food allergy testing was within normal limits. Serum IgE for latex was equivocal and a follow-up latex sensitivity test was performed and was within normal limits. Repeated skin biopsies were consistent with acute eosinophilic spongiotic dermatitis with vesicles most likely due to contact dermatitis. No therapy was initiated during the diagnostic period and no etiology was confirmed. Conclusions, Over time the dermatitis and eosinophilia resolved spontaneously. The animal is currently free of any lesions and maintains an eosinophil count within normal limits. [source] Oral pemphigus: long term behaviour and clinical response to treatment with deflazacort in sixteen casesJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 4 2000Michele D. Mignogna Abstract: Systemic corticosteroids remain the mainstay of therapy for pemphigus. Their use has transformed what was almost invariably a fatal illness into one whose mortality is now below 10%. Unfortunately, the high doses and prolonged administration of corticosteroids that are often needed to control the disease result in numerous side effects, many of which are serious or even life-threatening. Sixteen patients affected by oral pemphigus vulgaris were retrospectively examined to illustrate the natural course of the disease and to describe the efficacy of the treatment we utilised. Deflazacort, used with azathioprine, is the steroid of first choice in our therapeutic protocols, while cyclophosphamide and methylprednisolone "pulse therapy" are reserved for cases unresponsive to high doses of oral corticosteroids. In addition, the literature on oral pemphigus vulgaris was reviewed with respect to clinical history, signs and symptoms, management, and treatment outcome. [source] A study on plucked hair as a substrate for direct immunofluorescence in pemphigus vulgarisJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 2 2009M Daneshpazhooh Abstract Background, It has recently been demonstrated in a study on 15 patients that plucked hair can be used as a substrate for direct immunofluorescence (DIF) in pemphigus. Objective, Our aim was to assess the sensitivity of DIF on plucked hairs in pemphigus vulgaris (PV) patients with positive DIF of oral mucosa. Methods, One hundred and ten new PV patients were enrolled in the study. They all showed the typical clinical and histological findings as well as positive DIF of the oral mucosa, diagnostic for PV. Approximately 30 hairs were obtained in the same way as for the trichogram. The hairs with their outer root sheaths (ORS) were processed for DIF in order to detect immunoglobulin G and C3. Results, Immunodeposits favouring PV were demonstrated in the ORS of 100 cases showing a sensitivity of 91%. Conclusion, Regarding the relatively high sensitivity of DIF on plucked hair in PV patients with positive oral mucosal DIF in our study, it seems that hair plucking is a suitable alternative to the more invasive techniques of skin or mucosal biopsy for obtaining specimens for DIF in PV. Conflicts of interest None declared [source] Desmoglein 1 and 3 enzyme-linked immunosorbent assay in Iranian patients with pemphigus vulgaris: correlation with phenotype, severity, and disease activityJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 10 2007M Daneshpazhooh Abstract Background, Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder of the skin and mucosa characterized by the presence of autoantibodies against desmoglein3 (Dsg3). Some patients also have antibodies against desmoglein1 (Dsg1). The aims of this study were to evaluate the diagnostic value of Dsg enzyme-linked immunosorbent assay (ELISA) in Iranian PV patients, to assess its correlation with the clinical phenotype and severity of disease and to investigate the changes of these antibodies after treatment. Methods, Seventy-three patients with PV (29 men, 44 women) presenting to the Pemphigus Research Unit at Razi Hospital, Tehran, Iran were enrolled. ELISAs were used to detect IgG autoantibodies reactive with the ectodomains of Dsg1 and Dsg3, and the correlation of antibodies with the clinical phenotype as well as oral and skin disease severity was assessed. In addition, the tests were repeated in 18 patients after treatment and the resulting remission. Results, Anti-Dsg1 and anti-Dsg3 were detected in 56 (76.7%) and 69 (94.5%) patients, respectively. Anti-Dsg1 and anti-Dsg3 antibodies were present in 48 (94.1%) and 50 (98%) patients with mucocutaneous type, in 2 (12.5%) and 15 (93.7%) patients with mucosal type, and in 6 (100%) and 4 (66.7%) patients with cutaneous PV, respectively. The mean anti-Dsg1 index values were significantly higher in cutaneous and mucocutaneous phenotypes than mucosal PV (P < 0.001). The mean anti-Dsg3 index values were significantly lower in cutaneous and mucosal phenotypes than mucocutaneous PV (P < 0.01). The severity of skin lesions (but not oral lesions) was correlated with anti-Dsg1 antibody level (P < 0.001); on the other hand, the severity of oral lesions (P < 0.01) as well as skin lesions (P < 0.001) was significantly correlated with anti-Dsg3 antibody levels. Both anti-Dsg1 and anti-Dsg3 levels were significantly reduced after treatment and clinical remission (P < 0.001). Conclusion,, Dsg ELISA is not only a sensitive tool for the diagnosis of PV, it can also serve as a predictive means for assessing the severity as well as for monitoring the disease activity. Although, in general, the clinical phenotype is related to the antibody profile, there are occasional cases with discordant phenotype and antibody profile. These discrepancies might be explained by genetic variations or the presence of possible minor antigens involved in the pathogenesis of pemphigus. [source] Pemphigus vulgaris induced by radiotherapyJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 4 2004E Orion ABSTRACT Pemphigus can be triggered or induced by many insults, such as drugs, infections, viruses and neoplasms. X-ray-induced pemphigus has been recorded only rarely in the literature. We describe the case of a woman with pemphigus vulgaris in remission, who relapsed 1 week after completion of an irradiation course for breast cancer, in the exact location of the radiation. We review the previous 15 cases from the literature and outline the common features of those cases. [source] Dexamethasone pulse therapy in pemphigusJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2002Gg Tóth ABSTRACT Pulse therapy with high-dose glucocorticoids was introduced 20 years ago as a treatment modality for autoimmune disease and transplant rejection. The most popular dermatological indication for pulse therapy is severe pemphigus. We reviewed the sequelae of 14 patients with pemphigus who were treated by pulse therapy. Seven of them reached complete remission, although three of them needed a new pulse course due to disease flare-up. Adverse events were minor and confined to 60% of all patients: temporary facial flushing during pulse administration, sleep disturbances during the first night after pulse administration, and mood changes occurred during the week of pulse therapy. The study showed the possibility of oral instead of an intravenous route of dexamethasone pulse administration, which makes double-blind placebo-controlled trials ethically feasible. Fifty per cent of the patients reached complete remission. This retrospective study does not allow claims on the steroid-sparing effect. [source] | |||||||||||||||||||||||||||||||