Impact of smoking on pemphigus

Background  A positive history of smoking is less common in patients with pemphigus than in healthy subjects. The aim of this case–control study was to compare the remission rate and clinical locations involved in smokers and nonsmokers with pemphigus vulgaris.
Methods  Seventy patients with pemphigus vulgaris, treated with a uniform protocol, were enrolled. The sites of involvement, average time needed for disease control, and number of relapses were compared in smokers and nonsmokers. At the end of the first and second years of treatment, the rate of remission was compared in the two groups.
Results  Ten of the patients were current cigarette smokers, but the other 60 (85.7%) had no history of smoking. There was no difference in the rate of cutaneous or mucosal involvement between smokers and nonsmokers. The predominant subtype was the mucocutaneous type in both groups. Smokers with pemphigus vulgaris achieved partial remission more frequently than nonsmokers at the end of the first year of treatment. The number of patients in remission at the end of the second year of therapy was significantly higher for smokers with pemphigus than for nonsmokers. The main reason for disease activity in both groups was recurrence.
Conclusions  Cigarette smoking may not affect the rate of cutaneous or mucosal involvement in pemphigus; however, the data indicate that remission may be achieved sooner in pemphigus patients who smoke.     

Clinical and immunological features of pemphigus relapse

Pemphigus is a potentially fatal blistering skin disease. It is an autoimmune disease, meaning that the body's immune system, which normally fights off disease, in this case attacks healthy cells. The main treatments for pemphigus are with drugs called corticosteroids or immunosuppressive agents. The goal of treatment is to reach remission, a point where there are no new lesions (affected areas of skin) with minimal or no therapy (medication). However, many patients experience several relapses, meaning that after a period of improvement, the disease then worsens again, and it is often difficult for them to achieve remission. This study, from Japan, aimed to learn more about the risk factors and clinical features (signs) of pemphigus relapse. The authors retrospectively reviewed the medical records of 42 pemphigus patients. 61.9% of cases experienced relapse, usually when a medicine called oral prednisolone was tapered to around 0.1mg/kg. In a type of pemphigus called mucocutaneous pemphigus vulgaris (mcPV), the initial doses of prednisolone were lower in cases with relapse than without relapse. At relapse, mcPV shifted to subtypes (phenotypes) called mucosal dominant PV (mPV, which mainly affects the mouth and not the skin) (40%), pemphigus foliaceus (PF, affecting the skin) (20%) or others (20%). In contrast, the relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Looking at antibodies, which are produced by the immune system to help fight off disease, patients with both anti-Dsg1 and anti-Dsg3 antibodies to begin with, had recurrence with anti-Dsg3 antibodies alone (40%), with both anti-Dsg1 and Dsg3 antibodies (30%) or with anti-Dsg1 antibody alone (20%). In conclusion, when a patient with mcPV relapses, there can be changes in the phenotype of their disease, and the antibodies being produced. At least 1mg/kg/day of prednisolone, especially for mcPV cases, and prudent tapering around 0.1mg/kg may lead to better outcomes.     

Cutaneous type pemphigus vulgaris: a rare clinical phenotype of pemphigus

Pemphigus is an autoimmune blistering disease of the skin, mucous membranes, or both. There are two main categories of pemphigus: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). PV is further subdivided into mucosal dominant and mucocutaneous types, according to the extent of cutaneous lesions. These classes of pemphigus have distinct histopathologic and serologic findings, with most cases falling into these subtypes. We report 4 cases that clinically showed blisters and erosions in the skin only, without mucosal involvement. Histologic examination of cutaneous lesions demonstrated suprabasilar acantholysis, a typical finding for PV. These patients had predominant anti-desmoglein 1 (Dsg1) IgG autoantibodies as well as anti-Dsg3 IgG autoantibodies, as determined by enzyme-linked immunosorbent assay. The desmoglein compensation theory posits that this rare phenotype can be produced by pathogenically weak anti-Dsg3 IgG in the presence of potent anti-Dsg1 IgG autoantibodies. Thus, cutaneous type PV without apparent mucosal involvement is observed as a rare clinical and histologic expression of pemphigus. This expression can be a transient phenotype that may develop from, or evolve into, other subtypes of pemphigus.     

Value of direct immunofluorescence in predicting remission in pemphigus vulgaris

BACKGROUND: Pemphigus vulgaris is characterized by the presence of autoantibodies to desmogleins. Multiple relapses and remission may occur during the course of the disease. The goal of this study was to determine whether direct immunofluorescence study has any value in detecting immunological remission of pemphigus vulgaris. METHODS: Fifty-seven patients with pemphigus vulgaris who were in clinical remission for at least 3 months, while taking prednisolone 5-7.5 mg/day, were recruited retrospectively for the study. Direct immunofluorescence study had been performed in all patients after a period of at least 3 months in clinical remission. Treatment had been discontinued in all patients with negative results of direct immunofluorescence. RESULTS: Of 57 patients who were in clinical remission, 24 patients (42%) had negative and 33 patients (58%) had positive results of direct immunofluorescence. Eleven patients (46%) with negative results of direct immunofluorescence relapsed within the first year of the follow-up period. Nine patients with negative direct immunofluorescence had a history of more than 6 months of clinical remission before direct immunofluorescence study. Among them, two patients (22%) relapsed. None of four patients with history of more than 12 months of clinical remission before a negative direct immunofluorescence study relapsed. CONCLUSIONS: Negative direct immunofluorescence is an indicator of immunological remission in patients with pemphigus vulgaris after 6-12 months in clinical remission.     

Therapeutic effect of mizoribine on pemphigus vulgaris and pemphigus foliaceus

We evaluated the effectiveness of mizoribine, a newly developed immunosuppressive agent, as an adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. Eleven pemphigus patients (eight pemphigus vulgaris and three pemphigus foliaceus) received the combination therapy of prednisolone and mizoribine. Complete remission was observed in three of the eight patients with pemphigus vulgaris and in one of the three patients with pemphigus foliaceus. The four patients with complete remission had a rapid clinical response and achieved remission at a median of 11.8 months. Partial remission was achieved in two of the three patients with pemphigus foliaceus. The median time to achieve partial remission was 16.0 months. Six (55.6%) of the 11 patients with pemphigus had complete or partial remission and were able to taper their prednisolone. The cumulative probability of having a complete remission was 64.3% at 19 months of follow-up using Kaplan-Meier analysis. The effectiveness of the additional mizoribine therapy could be attributed to its corticosteroid-sparing properties as well as its immunosuppressive effects. The serum concentration titer of mizoribine was around 1.0?μg/mL 2 hours after administration. Patients who were not improved by the additional mizoribine might require a continuously higher dose of mizoribine to achieve effective therapy.     

Serum and salivary desmoglein 1 and 3 enzyme‐linked immunosorbent assay in pemphigus vulgaris: correlation with phenotype and severity

Background 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. Aims 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. Methods 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. Results 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). Conclusion Saliva Dsg ELISA could be used for diagnosis of PV. Salivary Dsg1 antibodies had a significant correlation with mucosal severity.     

Remisión clínica completa prolongada en pacientes con pénfigo vulgar grave después del tratamiento con ciclos intravenosos de ciclofosfamida

BackgroundCorticosteroids are the systemic treatment of choice in patients with pemphigus vulgaris, but chronic administration is associated with side effects. Intravenous treatment with cyclophosphamide can improve the clinical signs of pemphigus vulgaris.Material and methodsWe prospectively studied 8 patients diagnosed with pemphigus vulgaris. Six of these had mucocutaneous pemphigus vulgaris and 2 had mucosal pemphigus vulgaris. Treatment consisted of 10 cycles of cyclophosphamide at a dose of 10-15 mg/kg separated by 15 days, while maintaining the initial corticosteroid and immunosuppressant dose. Clinical efficacy was assessed and the anti-epidermal intercellular substance (EIS) and anti-desmoglein (DSG) 3 and 1 antibody titers were monitored (by indirect immunofluorescence and enzyme-linked immunosorbent assay, respectively).ResultsAll patients with pemphigus vulgaris responded excellently to treatment. Five of the 8 patients achieved complete remission of pemphigus lesions after 10 cycles of cyclophosphamide. In the other 3 patients, the skin lesions disappeared a few weeks after the last cycle of cyclophosphamide. A substantial reduction in immunosuppressant dose was possible in all patients. Furthermore, an improved immunologic response was observed in all cases after cyclophosphamide treatment, with decreased anti-DSG1 and anti-DSG3 antibody titers and well as decreased circulating anti-EIS antibody titers. During the mean 15.1 month follow-up (range, 1-25 months), no new lesions appeared and no side effects of cyclophosphamide therapy were reported.ConclusionsFortnightly cycles of intravenous cyclophosphamide may be a useful therapeutic option in patients with severe pemphigus vulgaris. A reduction of corticosteroid dose was possible with this therapeutic approach and the cumulative cyclophosphamide dose was lower than with daily oral administration. Our findings also show that the therapeutic approach induces clinical and immunologic remission in most patients.     

Pemphigus,analysis of 155 patients

Background Pemphigus vulgaris is a chronic autoimmune mucocutaneous blistering disease. In the last 20 years, immunomodulatory agents have been added to the therapeutic armamentarium. Only few recent studies have evaluated the long‐term outcome of pemphigus and possible prognostic parameters in a large group of patients. The aim of this study was to evaluate and analyse the course and prognostic factors of pemphigus in patients followed from 1976 to 2004. Patients and methods The study group consisted of 155 patients attending the pemphigus clinic of a major tertiary‐care medical centre. Background, clinical and treatment data were derived from the patient files and telephone contact. Statistical analysis was performed with Pearson correlation, Fisher exact test, and univariate and multivariate logistic regression models. Results Age < 40 years at disease onset, Sephardic Jewish origin, and mucosal involvement at diagnosis and high number of relapses were found to be independent prognostic factors of poor outcome. A long (> 1 year) primary remission was a good prognostic factor. During the 26‐year study period, 16 patients died. None of the deaths was directly related to either the disease or a complication of treatment. Conclusions The course and outcome of pemphigus are worse in patients who are young at diagnosis (< 40 years) or of Sephardic Jewish origin. Mucosal involvement at diagnosis and poor response to treatment also predict poor outcome. The mortality rate of pemphigus is apparently lower than reported in the literature, perhaps because of the contemporary use of adjuvant immunomodulatory therapeutic agents.     

The course and prognosis of pemphigus in 47 Tunisian patients

Background Pemphigus is a severe and life‐threatening autoimmune bullous dermatosis. Objective We have analysed parameters that may influence prognosis of pemphigus (P). Methods It was a retrospective study (2002–2010), with pemphigus considered as severe if body surface involvement ≥30%. Disease control and relapse‐free survival (Kaplan–Meier) were analysed and compared according to several parameters (P < 0.05). Results 47 cases of pemphigus were collected, mean age 51 years ± 16.8 (F/H = 3.27). There were 30 pemphigus profundus and 17 superficial pemphigus. The median remission period was of 9 months (1.2 months–5 years). The mean healing time was of 40 days (6 days–4 months), which did not depend on type of P, its severity or infectious complications, whereas it was shorter in aged patient (≥65 years) compared to non aged ones (P = 0.018). 36.2% of patients had relapsed. Relapses were significantly more frequently observed only in the presence of mucosal involvement at presentation (P = 0.015). The median overall 1st relapse‐free survival was of 2.33 years. Only mucosal involvement at presentation was associated with a shorter median 1st relapse‐free survival time (1.28 years vs. 3 years) (P = 0.0017). Mortality rate was of 10.6% (n = 5); in four patients the death was directly related to pemphigus and occurred rapidly after the onset of lesions. Conclusion Our study illustrates the poor prognosis of pemphigus by a long duration to disease control, a high initial dose of oral steroid, a high rate of relapse and a short remission period. Only mucosal involvement at presentation was identified as a poor prognostic factor.     

Cyclophosphamide pulses with oral prednisolone in the treatment of pemphigus: a pilot study

An open labeled clinical trial aimed at assessing the efficacy and safety of pulse intravenous cyclophosphamide with daily oral prednisolone in the treatment of pemphigus was carried out. Twenty-six patients (12 men, 14 women; mean age, 48.4 years), comprising 25 cases with pemphigus vulgaris and 1 with pemphigus vegetans (< 10% body surface area involvement) who did not achieve adequate control on corticosteroids with or without other adjuvants were included. After baseline evaluation, monthly intravenous boluses of cyclophosphamide (15 mg/kg) along with daily oral prednisolone (starting dose 1 mg/kg/day, tapered according to clinical response) were administered. Patients were assessed monthly for clinical activity and side-effects. All patients experienced significant clinical improvement within 1 month of starting treatment. Healing of skin and mucosal lesions occurred respectively at mean durations of 2.1 and 3.6 months. Three weeks to 8 months later, 9 patients had recurrences of activity on tapering/withdrawal of prednisolone, mainly in the oral mucosa. Side effects of treatment included amenorrhea (3 patients), microscopic hematuria (3) which cleared with co-administration of mesna, vomiting (1), weight gain (10), gastritis (1), and cataract (2). It is concluded that treatment with monthly intravenous cyclophosphamide boluses along with daily oral prednisolone clears lesions of pemphigus with < 10 percent body surface involvement, and this may be an alternative regimen for pemphigus. Monitoring for adverse effects is essential.     

Rituximab therapy for refractory autoimmune bullous diseases: A multicenter,open‐label,single‐arm,phase 1/2 study on 10 Japanese patients

This was a multicenter study of rituximab, a chimeric monoclonal immunoglobulin G antibody directed against CD20, for the treatment of refractory autoimmune bullous diseases (pemphigus and pemphigoid). Ten patients (three with pemphigus vulgaris, six with pemphigus foliaceus and one with bullous pemphigoid) were treated with a single cycle of rituximab (four weekly infusions at a dose of 375 mg/m² of body surface area). The primary end‐points were the number of serious adverse events and rate of complete remission at 40 weeks. Five patients (50%) achieved complete remission with minimal therapy (defined as no active lesions with lower doses of systemic corticosteroids compared to that with prednisolone 10 mg/day). Improvements in clinical scores (Pemphigus Disease Area Index) and decreases in autoantibody titers in the sera were observed in the four pemphigus patients who failed to achieve complete remission. This suggests that rituximab was effective in nine of 10 cases. Two serious adverse events (Pneumocystis carinii pneumonia and septic shock due to infectious arthritis) were observed and adequately treated with hospitalization. CD19‐positive B lymphocytes in the peripheral blood decreased on day 29 following rituximab treatment, and remained at low levels throughout the observation period (280 days). Our results confirmed the efficacy of rituximab therapy for refractory autoimmune bullous diseases in Japan.     

Anti-desmoglein IgG autoantibodies in patients with pemphigus in remission

Background   Desmoglein (Dsg) enzyme-linked immunosorbent assay (ELISA) is a highly sensitive and specific method to detect anti-Dsg3 and anti-Dsg1 IgG autoantibodies in pemphigus vulgaris (PV) and pemphigus foliaceus (PF), respectively. Whereas ELISA index values fluctuate in parallel with disease activity, ELISA positivity during clinical remission has been observed.
Objective   To determine the prevalence of positive Dsg ELISA index values during clinical remission. To ascertain how positive Dsg ELISA scores during remission compare with those during active disease.
Methods   Dsg ELISA was performed on serum samples of PV and PF patients taken during remission (lesion-free ≥ 3 months on ≤ 15 mg or ≤ 5 mg/day prednisolone) and active disease. We used a modified ELISA protocol with optimal serum dilutions in sera with very high initial index values, as we previously described.
Results   When remission was defined as no eruption ≥ 3 months with ≤ 15 mg/day prednisolone, 20 of 43 PV patients (46.5%) and 4 of 12 PF patients (33.3%) showed Dsg3 and Dsg1 ELISA positivity, respectively. With ≤ 5 mg/day, 6 of 17 PV (35.3%) and 1 of 6 PF patients (16.7%) showed Dsg3 and Dsg1 ELISA positivity, respectively. The index value of each ELISA-positive remission serum was consistently lower than that of its corresponding active disease serum. We observed consistent correlation between ELISA index values and indirect immunofluorescence titres.
Conclusions   Circulating anti-Dsg IgG autoantibodies are found in a considerable percentage of pemphigus patients in remission, who have high levels of antibody production during active stages.     

Five Japanese cases of antidesmoglein 1 antibody-positive and antidesmoglein 3 antibody-negative pemphigus with oral lesions

Background Oral mucosal lesions develop in pemphigus vulgaris, but not in pemphigus foliaceus. This clinical phenomenon is explained by the ‘desmoglein (Dsg) compensation theory’. Dsg3 and Dsg1 are major autoantigens for pemphigus vulgaris and pemphigus foliaceus, respectively. Dsg3 is overexpressed and Dsg1 is weakly expressed on the oral mucosa. Thus, on the oral mucosa, suppression of Dsg3 function by anti‐Dsg3 autoantibodies is not compensated by weakly expressed Dsg1 in pemphigus vulgaris, while suppression of Dsg1 function by anti‐Dsg1 autoantibodies is perfectly compensated by richly expressed Dsg3 in pemphigus foliaceus. Objectives We present five Japanese patients with pemphigus who deviate from this theory, i.e. all patients showed oral lesions (three also had cutaneous lesions) and reacted only with Dsg1, but not with Dsg3, by enzyme‐linked immunosorbent assay. Methods To confirm whether the unique clinical phenotypes in our patients were due to a different immunological profile from that in classical pemphigus, we examined the reactivity of the patient sera by immunoprecipitation‐immunoblotting analysis using five Dsg1/Dsg2 domain‐swapped molecules. Results The sera of two patients who had only oral lesions tended to react with the extracellular (EC) 5 domain of Dsg1, the domain that is considered nonpathogenic in classical pemphigus foliaceus. Sera of three patients with mucocutaneous lesions reacted with EC1 domain or with both EC1 and EC2 domains of Dsg1, like classical pemphigus foliaceus. Conclusions These results indicate that antigenic diversity of anti‐Dsg1 antibodies in these patients may cause the unique oral mucosal and cutaneous lesions, although further studies are required to elucidate the pathomechanisms.     

Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus

Paraneoplastic pemphigus (PNP) is a rare, life‐threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus‐like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological‐related neoplasms in 11 patients, with Castleman's disease (n = 4) as the most frequent. Twelve patients were followed for 5–148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2‐year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.     

Randomized controlled trial of adjuvant oral dexamethasone pulse therapy in pemphigus vulgaris: PEMPULS trial

OBJECTIVE: To determine the therapeutic effect of adjuvant dexamethasone pulse therapy when given in addition to conventional treatment of pemphigus vulgaris. DESIGN: A randomized, placebo-controlled trial. SETTING: International European, multicenter outpatient and inpatient study. PATIENTS: Of the 20 enrolled patients, 11 were randomized to the dexamethasone pulse (DP) group and 9 to the placebo pulse (PP) group. INTERVENTIONS: Oral dexamethasone in 300-mg pulses or PPs 3 days per month. During the intervention, the DP and PP groups received conventional treatment with prednisolone, 80 mg/d, which was tapered across 19 weeks, and azathioprine sodium, 3 mg/kg per day, until the end of the study. Monthly pulses were continued until prednisolone treatment was tapered to 0 mg. MAIN OUTCOME MEASURES: Number of patients in remission, time to and duration of remission, cumulative prednisolone dose, and occurrence of adverse events during 1 year of follow-up. RESULTS: Eight of the 11 DP-treated patients and all 9 PP-treated patients achieved remission. Mean time to remission was 173 days with DP and 176 days with PP. The mean duration of remission within the first year was 151 days for DP and 141 days for PP. Mean cumulative prednisolone dose was 5300 mg for DP and 4882 mg for PP. Weight gain (>5% of baseline) occurred in 8 DP-treated patients compared with 1 PP-treated patient (P<.01). We found no statistically significant difference (P>.05) of an adjuvant effect of DP on remission of pemphigus vulgaris. CONCLUSION: In patients with new pemphigus vulgaris disease activity, there was no benefit of oral DP therapy given in addition to conventional treatment. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00127764.     

A Clinicopathological Study of Pemphigus in Eastern India with Special Reference to Direct Immunofluorescence

Background:Pemphigus is a group of chronic autoimmune vesico-bullous disorders in which the epidermis and the basement membrane zone are the focus of attack resulting in cutaneous and mucosal blister formation. Direct immunofluorescence (DIF) test is a very sensitive test for the diagnosisAim:To study the clinico histopathological patterns of pemphigus in eastern India. The study also aims to correlate DIF with clinical and histologic findings as well as severity of skin involvement [scoring systems].Results:In our study Pemphigus vulgaris (PV) was the predominant type with 32 cases followed by 8 cases of pemphigus foliaceus (PF) and a single case of IgA pemphigus. Mean age at presentation was late middle age. Majority of the patients, 26 (63.41%) initially had cutaneous involvement followed by mucosal involvement. In this study group 36 (87.80%) patients showed acantholytic cells on histopathological examination. Most patients of PV showed suprabasal blister 20 (62.50%) followed by intraspinous 5 (15.62%) and subcorneal 5 (15.62%) blister. In majority 28 (87.50%) of the PV patients IgG and C3 antibodies were deposited throughout the epidermis. The strength of antibody positivity was strong in most of the patients (71.87%). In cases of PF mostly IgG 6 (75%) antibodies were deposited in the upper epidermis. DIF intensity had poor correlation with disease activity/severity except in PF.Conclusion:Almost 85.36% cases of pemphigus were diagnosed clinicopathologically. But 6 cases couldn’t be diagnosed accurately on clinicopathological basis and in them DIF was confirmatory. Two cases of pure mucosal PV and 1 case of IgA pemphigus was confirmed by DIF. Two cases of bullous pemphigoid clinico-histologically mimicking PV were also excluded by DIF. So it appears from our study that DIF is confirmatory for diagnosis of pemphigus in all cases.     

Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil

BACKGROUND: Mycophenolate mofetil is increasingly being used as a corticosteroid-sparing agent in immunosuppressive regimens. OBJECTIVE: To elucidate the effectiveness of mycophenolate as adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. DESIGN: Historical prospective study. SETTING: University hospital. PATIENTS: The study included 42 consecutive patients with pemphigus (31 with pemphigus vulgaris and 11 with pemphigus foliaceus) who had relapses during prednisone taper or had clinically significant adverse effects from previous drug therapy. RESULTS: Remission was achieved in 22 (71%) and 5 (45%) of patients with pemphigus vulgaris and pemphigus foliaceus, respectively. Partial remission was achieved in 1 (3%) and 4 (36%), respectively. The median time to achieve complete remission was 9 months (range, 1-13 months). The treatment was administered for a median of 22 months, and the median follow-up period was 22 months. Seventy-seven percent of patients had no adverse effect. Two patients had side effects severe enough to necessitate discontinuation of treatment, one because of symptomatic but reversible neutropenia and the other because of nausea. CONCLUSION: Mycophenolate is an effective and safe adjuvant in the treatment of both pemphigus vulgaris and pemphigus foliaceus.     

天疱疮特异性抗体亚型与疾病活动的相关性研究

目的 探讨天疱疮患者抗桥粒芯糖蛋白（Dsg）1和Dsg3抗体亚型与疾病活动的相关性。方法 收集47例天疱疮患者血清,ELISA检测其特异性抗Dsg1和Dsg3抗体及亚型,分析抗体滴度及亚型与疾病活动的相关性。结果 抗Dsg1和Dsg3抗体类别与天疱疮临床类型有关,17例皮肤黏膜同时受累的患者中有14例（82.4%）同时存在两种抗体;16例仅皮肤受累的患者中有15例（93.7%）存在抗Dsg1抗体,仅1例（6.3%）存在抗Dsg3抗体;6例仅黏膜受累的患者只存在抗Dsg3抗体,阳性率为100％。随着病情的加重,抗Dsg1和Dsg3抗体滴度有上升趋势,但该趋势与疾病的严重度并不完全平行。而抗体亚型则和疾病活动相关,活动期以特异性IgG4亚型为主,稳定期以IgG1亚型为主。抗Dsg1阳性者,活动期特异性IgG4和IgG1抗体吸光度（A）值分别为1.92 ± 1.21和0.60 ± 0.61,IgG4/IgG1 > 1;稳定期特异性IgG4和IgG1抗体A值分别为0.03 ± 0.02和0.22 ± 0.11,IgG4/IgG1 < 1;抗Dsg3阳性者,活动期特异性IgG4和IgG1抗体A值分别为2.35 ± 2.17和1.84 ± 1.16,IgG4/IgG1 > 1;稳定期特异性IgG4和IgG1抗体A值分别为0.15 ± 0.16和1.05 ± 0.77,IgG4/IgG1 < 1。结论 天疱疮特异性抗体亚型与疾病活动密切相关,用ELISA检测天疱疮患者血清中抗Dsg1和Dsg3抗体类型、特异性抗体亚型及滴度,可更好地辅助诊断疾病、监测疾病活动。     

Comparative efficacy of thalidomide and prednisolone in the treatment of moderate to severe erythema nodosum leprosum: A randomized study

The present study was undertaken to compare the efficacy and safety of thalidomide to that of oral prednisolone in the treatment of moderate to severe type 2 lepra reaction. Sixty patients with a histologically confirmed diagnosis of erythema nodosum leprosum with a clinical score of 4 or more (i.e. moderate to severe type 2 reaction) were randomly allocated to two groups comprising 30 patients each. Group 1 patients were given thalidomide at a dose of 300 mg/day for 1 week and the dose was gradually reduced, and Group 2 received prednisolone 40 mg daily for 2 weeks, which was tapered by 10 mg every 2 weeks. Thalidomide induced a faster clinical response (cutaneous as well as systemic) compared with prednisolone. Patients taking thalidomide had fewer relapses and a longer period of remission than those receiving prednisolone.     

ABSIS评分在寻常型天疱疮患者中的应用

目的 探讨自身免疫性大疱性皮肤病严重程度评分(ABSIS评分)在寻常型天疱疮治疗中的应用价值。方法 对本科的16例寻常型天疱疮入院时、激素减量时及出院时应用ABSIS评分法进行皮肤及黏膜严重程度评分,并同时记录患者的抗天疱疮抗体滴度。结果 入院时、糖皮质激素减量时及出院时ABSIS皮肤评分中位数分别为55,36及15.5,平均52.8,34.3和15.4,ABSIS黏膜评分中位数分别为3/16,2/8.5及0/0,平均3.3/15.8,2.1/8.3和0.6/1.1。抗天疱疮抗体滴度中位数分别为1:160,1:80及1:40,平均1:387.5,1:141.3及1:55。结论 ABSIS评分可从患者的皮损面积及质量、受累口腔黏膜数量及质量方面全面评价天疱疮患者的病情,比监测抗天疱疮抗体滴度能更灵敏的反应病情变化情况,可用于指导寻常型天疱疮患者的治疗及护理。