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ABSTRACT: Background The classic treatment for pemphigus vulgaris is prednisolone. Immunosuppressive drugs can be used in association. Objective To compare the efficacy of Azathioprine in reducing the Disease Activity Index (DAI). Patients and methods A double blind randomized controlled study was conducted on 56 new patients, assigned to two therapeutic groups: (i) prednisolone plus placebo; (ii) prednisolone plus Azathioprine. Patients were checked regularly for 1 year. 'Complete remission' was defined as healing of all lesions after 12 months, and prednisolone <7.5 mg daily, (DAI ≤ 1). Analysis was done by 'Intention To Treat' (ITT) and 'Treatment Completed Analysis' (TCA). Results Both groups were similar in age, gender, disease duration, and DAI. Primary endpoint: By ITT and TCA, the mean DAI improved in both groups with no significant difference between them. The difference became significant for the last trimester (3 months; ITT: P = 0.033, TCA: P = 0.045). Secondary endpoint: The total steroid dose decreased significantly in both groups, with no significant difference between them, except for the last trimester (ITT: P = 0.011, TCA: P = 0.035). The mean daily steroid dose decreased gradually in both groups becoming statistically significant in favour of azathioprine, in the last trimester, especially at 12th months (ITT: P = 0.002, TCA: P = 0.005). Complete remission was significant at 12 months only for TCA (AZA/Control: 53.6%/39.9%, P = 0.043). Limitations Sample size was rather small to demonstrate all differences. Other limitations include the choice of primary and secondary endpoints and the unavailability to measure thiopurine methyltransferase activity. Conclusion Azathioprine helps to reduce prednisolone dose in long-run.
Journal of the European Academy of Dermatology and Venereology 10/2012; · 2.98 Impact Factor
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ABSTRACT: Methotrexate (MTX) is a well-known systemic drug for moderate to severe chronic plaque psoriasis. Recently, mycophenolate mofetil (MMF) has been recommended for psoriasis.
To compare the efficacy and safety of MMF vs. MTX for the treatment of chronic plaque psoriasis.
Thirty-eight consecutive patients with Psoriasis Area and Severity Index (PASI)>10 were randomly assigned for 12 weeks of treatment with either MTX (18 patients; initial dose, 7.5 mg/week) or MMF (20 patients; dose; 2 g/day) and were followed for 12 weeks after discontinuing the treatment. The differences between the two groups were analysed at the end of treatment and follow-up comparing with baseline values.
After 12 weeks of treatment, the mean ± SD score for the PASI decreased from 16.46 ± 5.29 at baseline to 3.17 ± 2.35 among 15 patients treated with MTX, whereas the score decreased from 17.43 ± 7.42 to 3.97 ± 5.95 among 17 patients treated with MMF (P>0.05). Twelve weeks after discontinuing the treatment, the scores were 4.77 ± 3.52 and 5.94 ± 4.27, respectively (P>0.05). PASI -75 were achieved in 58.8% of patients in MMF group and 73.3% in MTX group (P > 0.05). Three months after discontinuing the treatment, PASI-75 remained in 33.3% of patients in MMF and 53.3% of MTX group (P > 0.05). Both drugs were well tolerated and side-effects were minor and transient.
No significant differences in efficacy were found between MTX and MMF groups. MMF may represent a good alternative for the treatment of psoriasis in patients who are unable to take MTX or other available drugs due to contraindication or toxicity.
Journal of the European Academy of Dermatology and Venereology 04/2010; 24(12):1447-51. · 2.98 Impact Factor
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ABSTRACT: To the best of our knowledge there is only one report about salivary desmoglein (Dsg) 1 and 3 enzyme-linked immunosorbent assay (ELISA) in pemphigus vulgaris (PV), whereas several studies have been performed on serum.
To find the sensitivity of serum and salivary anti-Dsg1 and 3 antibodies in the diagnosis of PV, and to determine the relationship between disease severity and phenotype with antibody levels.
Fifty new patients with PV were included in this study. The diagnosis of PV was confirmed by histopathology and direct immunofluorescence. Demographical data, disease severity and phenotypes were recorded on questionnaire sheets. Dsg1 and Dsg3 ELISA were performed on serum and salivary samples of patients and controls.
Thirty-seven patients had mucocutaneous phenotype; whereas mucosal dominant and cutaneous dominant phenotypes were seen in 11 and 2 patients respectively. The sensitivities of serum anti-Dsg3 and anti-Dsg1 were 94% and 72% respectively. The sensitivities of salivary anti-Dsg3 and anti-Dsg1 antibodies were accordingly 94% and 70%. Compared with mucosal phenotype, serum and salivary anti-Dsg1 antibodies were significantly higher in the patients with mucocutaneous phenotype. Serum Dsg1 antibodies were related with cutaneous and serum Dsg3 antibodies with mucosal severity scores. Salivary Dsg1 antibodies were significantly correlated with mucosal severity (P=0.00); however there was no correlation between this antibody and cutaneous severity (P=0.07). Salivary Dsg3 antibodies were not correlated with mucosal severity (P=0.16).
Saliva Dsg ELISA could be used for diagnosis of PV. Salivary Dsg1 antibodies had a significant correlation with mucosal severity.
Journal of the European Academy of Dermatology and Venereology 09/2009; 24(3):275-80. · 2.98 Impact Factor
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ABSTRACT: Pemphigus vulgaris (PV) usually presents as non-healing, painful oral erosions, but transient or aphtha-like lesions are not exceptional at the very beginning of the disease leading to the common misdiagnosis of recurrent aphthous stomatitis (RAS). We designed this cross-sectional, questionnaire-based study to evaluate this underreported yet important presentation of PV.
One hundred and eighty-five consecutive PV patients were interviewed and a questionnaire, comprising items related to the natural history of oral lesions, was filled in for each. Fourteen patients who had taken steroids (topical or systemic) before their final diagnosis were excluded.
Twenty-three per cent of patients gave a history of transient aphthous-like lesions; 95% of them were misdiagnosed as aphthae. These lesions were especially reported by patients aged 40 years or older (P < 0.047).
PV should be kept in mind as a rare differential diagnosis of transient oral ulcerations. We recommend careful observation of these patients and performing indirect immunofluorescence or desmoglein ELISA and even biopsy in atypical cases, to rule out PV especially in older patients and predisposed ethic groups. To find out the differential aspects of RAS and aphthous-like PV, a cohort study on RAS patients is suggested.
Journal of the European Academy of Dermatology and Venereology 09/2008; 23(2):157-9. · 2.98 Impact Factor
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ABSTRACT: 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.
Our aim was to assess the sensitivity of DIF on plucked hairs in pemphigus vulgaris (PV) patients with positive DIF of oral mucosa.
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.
Immunodeposits favouring PV were demonstrated in the ORS of 100 cases showing a sensitivity of 91%.
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.
Journal of the European Academy of Dermatology and Venereology 09/2008; 23(2):129-31. · 2.98 Impact Factor
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ABSTRACT: Pemphigus vulgaris can be divided into mucosal, mucocutaneous and cutaneous subtypes. A higher mortality rate has been shown with mucocutaneous involvement.
The aim of this retrospective study was to analyse the association of clinical subtypes of pemphigus vulgaris with remission rates.
One hundred twenty-eight patients with pemphigus vulgaris, treated with prednisolone 2 mg/kg/day plus azathioprine 2 to 2.5 mg/kg/day, were enrolled. The partial and complete remission rates, at the end of the first and second years of treatment, and the number of relapses were compared in the three groups.
After disease establishment, 71.1% had mucocutaneous, 18.8% had mucosal, and 10.2% had only cutaneous involvement. The mean duration of follow-up was 53.5 +/- 39.6 months. The mean duration it took the mucocutaneous group to reach a prednisolone dosage of 30 mg/day was significantly longer (P = 0.050). Mucocutaneous patients had a significantly lower rate of remission (31.9%) compared with those with only mucosal or cutaneous involvement (48.6%) at the end of the first year of the treatment (P = 0.029). After 2 years, mucocutaneous patients again had a lower remission rate (32.9% vs. 44.5%). Relapses were also more frequent in this subtype. Those presenting with mucosal or mucocutaneous erosions had a higher rate of active disease after receiving treatment for a year compared with those with only cutaneous presentation (66.7% vs. 45%; P = 0.057).
In mucocutaneous subtype, clinical control was achieved later, and they had a lower rate of remission at the end of the first and second years of treatment. They were also prone to relapses.
Journal of the European Academy of Dermatology and Venereology 06/2008; 22(5):580-4. · 2.98 Impact Factor
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Journal of the European Academy of Dermatology and Venereology 05/2008; 22(4):526-7. · 2.98 Impact Factor
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ABSTRACT: Pemphigus vulgaris (PV) is an autoimmune blistering disease which is known to involve the female genital tract, but the frequency at which this occurs is unknown. There are few reports in the literature of the cytological appearance of PV on cervicovaginal smears.
To evaluate involvement of the female genital tract and the appearance of cervicovaginal Papanicolaou (Pap) smears in PV.
The study included 77 patients with PV who attended between April 2005 and February 2007. Each patient was subjected to gynaecological examination and cervicovaginal Pap smear.
Genital lesions were observed in 39 patients (51%). Of these, the labia minora were involved in 36 patients (92%), the labia majora in 11 (28%), the vagina in 14 (36%) and the cervix in six (15%). Cervicovaginal Pap smears of 20 of 77 patients (26%) showed PV. Of 72 satisfactory Pap smears, the cervical Pap smear was normal in 25 patients (35%), inflammatory in 43 patients (60%), and dysplastic (low-grade squamous intraepithelial neoplasia) in four patients (6%).
Involvement of the female genital tract with PV might not be as infrequent as was previously thought; it is probably the second most common mucosal site of PV after the oral mucosa. Genital lesions may be missed and the need for thorough pelvic examination should not be overlooked.
British Journal of Dermatology 04/2008; 158(3):478-82. · 3.67 Impact Factor
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ABSTRACT: 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.
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.
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).
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.
Journal of the European Academy of Dermatology and Venereology 11/2007; 21(10):1319-24. · 2.98 Impact Factor
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ABSTRACT: Recent reports have revealed the relatively high incidence of pemphigus in Iran. Occupational exposure and personal habits have been suggested to play a role in the aetiopathogenesis of this life-threatening disease.
In order to analyse the association of environmental factors with pemphigus, we conducted a case-control study to evaluate the possible role of smoking, pesticide exposure and hormonal factors in Iran.
This study was conducted in Iran using a structured questionnaire. Questions included information on patients' smoking habits, occupational exposure to pesticides, use of oral contraception (OC) and number of pregnancies.
We enrolled 210 patients with pemphigus and 205 control subjects. Fewer of patients with pemphigus (17.1%) reported a current or past history of smoking, which was statistically different from the control group (27.3% smokers). The duration of smoking and the number of cigarettes smoked daily was also significantly lower in patients. Although OC use was significantly higher in women with pemphigus, the mean number of pregnancies was not different between the two groups. Occupational exposure to pesticides was significantly higher in patients with pemphigus (14.8%) than in controls (5.4%); patients with pemphigus were exposed to pesticides three times more often than were healthy subjects.
As a positive history of smoking was lower in patients with pemphigus compared with healthy subjects, it seems that smoking is a protective factor in pemphigus. This should encourage further investigations, searching for novel therapies. If pesticides and OC are confirmed as triggering factors, their cessation might reduce the need for pharmacological therapy.
Clinical and Experimental Dermatology 06/2007; 32(3):256-60. · 1.20 Impact Factor
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ABSTRACT: Pemphigus vulgaris (PV) is a severe blistering disease involving the skin and mucous membranes. The most common causes of death in these patients are adverse effects of drugs, and infection. Skin lesions are one of the important sources of infection. Thus, any local treatment that could reduce healing time of lesions and consequently reduce the total dosage of drugs needed to treat is favourable.
To evaluate the efficacy of epidermal growth factor (EGF) in reducing healing time of lesions in patients with pemphigus vulgaris.
In this randomized, double-blind, within-patient, left/right, controlled trial, 20 hospitalized patients with pathologial and immunohistologial (direct and indirect immunoflourecence) proven pemphigus vulgaris (PV) were chosen. In addition, all patients had at least one appropriate pemphigus lesion on each side of the body that had not healed after 2-week systemic therapy and sterile saline washing. EGF (10 microg/g) in 0.1% silver sulfadiazine cream vs. 0.1% silver sulfadiazine cream alone was applied randomly on one side of the body.
Kaplan-Meier survival analysis suggested that median time to heal with application of EGF plus silver sulfadiazine cream was 9 days, in comparison with 15 days for silver sulfadiazine cream alone (log-rank test, P=0.0003). No intervention-related adverse effect was observed during the study.
EGF can significantly reduce healing time of skin lesions in patients with pemphigus vulgaris, at least when this cream base is applied (Cochrane skin group identifier: CSG20).
Journal of the European Academy of Dermatology and Venereology 02/2007; 21(1):79-84. · 2.98 Impact Factor
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ABSTRACT: Although early stage mycosis fungoides (MF) has a generally good prognosis, and long-term survival rates with current therapies (UVB, photochemotherapy, topical nitrogen mustard, electron beam radiotherapy) are similar, there is concern regarding their potential side effects. It has been reported that the same effective UVB dose is safer than PUVA in terms of carcinogenicity, and that it produces fewer side effects. Our aim was to evaluate the effect of narrow-band UVB in the treatment of early stage MF. Sixteen patients (seven males, nine females; mean age, 40 years) with early stage MF received TL-01 phototherapy three times per week using a standard protocol. Twelve patients (75%) had complete response in a mean of 27.9 treatments, three had partial response, and one no response. Upon discontinuation of treatment, six patients with complete response relapsed in a mean time to relapse of 4.5 months. The present study indicates that narrow-band-UVB is an effective treatment modality for early stage MF.
Clinical and Experimental Dermatology 08/2005; 30(4):376-8. · 1.20 Impact Factor
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ABSTRACT: The severe involvement, in one of our cases, prompts to be careful in the manipulation of PG like lesions. The only successful
treatment in the two cases was cyclosporin.
12/2003: pages 337-338;
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ABSTRACT: Clinically it is impossible to differentiate oral aphthosis of BD from that of RAS. There is a high resemblance between the
two entities. Positive pathergy test and HLA-B51 are the main differentiating factors.
12/2003: pages 317-320;
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C Chams-Davatchi
Advances in experimental medicine and biology 02/2003; 528:329-30. · 1.09 Impact Factor
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Advances in experimental medicine and biology 02/2003; 528:317-20. · 1.09 Impact Factor
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Advances in experimental medicine and biology 02/2003; 528:337-8. · 1.09 Impact Factor
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ABSTRACT: Pemphigus vulgaris is a severe autoimmune blistering disease of the skin and mucous membranes. In absence of treatment, mortality is high. In the past, prednisolone was the best treatment. Later, combination therapy with systemic prednisolone and other disease-modifying drugs was tried with better results. Unfortunately, some patients do not respond well to such treatment, or may exhibit multiple recurrences or complications. Some other patients may remain on high dose corticosteroids to maintain remission.
To evaluate the efficacy and safety of mycophenolate mofetil as a steroid sparing agent in the treatment of resistant pemphigus vulgaris.
We administered 2 g daily mycophenolate mofetil with systemic steroids to 10 patients with resistant and severe disease who did not respond to conventional therapy, or had multiple recurrences.
Nine of the ten patients responded to treatment and showed complete clearance of lesions within 6 to 16 weeks of therapy. At the end of six months, the dose of prednisolone was significantly lower. Side effects were few and mild. After discontinuation of mycophenolate mofetil, 5 of the 9 patients relapsed.
Mycophenolate mofetil is effective and safe as a disease-modifying drug combined with prednisolone in the treatment of patients with resistant pemphigus vulgaris. To induce long lasting remission has to be administrated for more than 6 months.
Annales de Dermatologie et de Vénéréologie 02/2002; 129(1 Pt 1):23-5. · 0.72 Impact Factor
