Pemphigus in Korea: clinical manifestations and treatment protocol

Pemphigus, a rare, chronic blistering disease of the skin and mucous membranes with severe morbidity and occasional mortality, is the most common autoimmune bullous disease in Korea. The purpose of this study was to evaluate the clinical features and propose a treatment strategy for patients with pemphigus. A retrospective analysis was conducted of 51 pemphigus patients seen between 1993 and 2001. Pemphigus vulgaris (PV) was the most common type with 32 cases, followed by 19 cases of pemphigus foliaceus (PF). The male to female ratio was 1:1.3, with females predominating, particularly among PV patients (PV, 1:1.5; PF, 1:1.1). The average ages at onset of PV and PF were 44.3 and 51.0 years old, respectively. Mucosal involvement was noted in 27 cases (84.4%) of PV but in only 3 cases (15.8%) of PF. Most patients initially received relatively low to intermediate doses (0.3-1.0 mg/kg/day) of prednisolone, and 23 (71.9%) PV patients and 10 (52.6%) PF patients also received immunosuppressive agents. Oral prednisolone and azathioprine (100 mg/day) formed the mainstay of treatment for our patients (47.1%). At the time of writing, 25.5% (13/51) of patients are in complete remission, and 72.5% (37/51) are undergoing maintenance therapy. One patient died due to sepsis during the treatment. For the treatment of pemphigus, a course of the lowest possible corticosteroid dosage in combination with immunosuppressive agents appears to be effective and less toxic than a high corticosteroid dosage.     

Successful treatment of autoimmune bullous diseases with a combination therapy of ciclosporin and corticosteroid

Three patients with autoimmune bullous diseases, pemphigus vulgaris, pemphigus foliaceus and bullous pemphigoid, were treated with a combination therapy of ciclosporin and corticosteroid. These patients responded to systemic low-dose prednisolone (or dexamethasone) and low-dose ciclosporin therapy; the result was prolonged complete remission. The addition of low-dose ciclosporin may produce enhanced clinical effects of steroid therapy without increasing any significant side effects.     

Rituximab in autoimmune bullous diseases: mixed responses and adverse effects

BACKGROUND: Intolerably high doses of systemic corticosteroids and additional immunosuppressants may be required to control disease activity in autoimmune bullous skin diseases. New therapeutic options are needed for such patients. OBJECTIVES: To determine the efficacy and adverse effects of adjuvant rituximab. METHODS: Seven patients with refractory autoimmune blistering diseases (pemphigus vulgaris, PV, n = 4; bullous pemphigoid, BP, n = 2; mucous membrane pemphigoid, MMP, n = 1) were treated four times with rituximab at an individual dose of 375 mg m(-2) at weekly intervals. RESULTS: All lesions cleared in three patients (two PV, one BP), while they were reduced by more than 50% in three others (two PV, one BP). The concomitant immunosuppressive medication was reduced in five patients (four PV, one BP). The patient with MMP developed bilateral blindness while nasopharyngeal lesions resolved. Three patients (two BP, one PV) experienced severe adverse events including fatal pneumonia. CONCLUSIONS: Adjuvant B-cell depletion by rituximab is effective in otherwise therapy-resistant bullous autoimmune disorders but may be associated with substantial adverse effects including fatal outcomes.     

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.     

Dual diagnosis of Pemphigus and pemphigoid. Retrospective review of thirty cases in the literature.

BACKGROUND: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. OBJECTIVE: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. METHODS: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. RESULTS: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. CONCLUSION: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.     

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.     

Spectrum of autoimmune bullous diseases in Iran: a 10-year review

Background Autoimmune bullous diseases (ABDs) are potentially devastating bullous dermatoses of the skin and mucosae characterized by the presence of tissue‐bound and circulating antibodies directed against disease‐specific target antigens. These diseases comprise two major subgroups of subepidermal autoimmune bullous disorders and pemphigus, based on the level of blister formation. Although they occur worldwide, the relative frequencies of different ABDs show wide geographical variation. Most epidemiological studies on ABDs have focused on single diseases or a group of diseases; published surveys studying the whole spectrum of diseases are scarce. Objectives This study aimed to assess the relative frequencies of different ABDs in patients presenting to the Bullous Diseases Research Center, Tehran, Iran. Methods Medical files for all newly diagnosed patients with ABD presenting to the Center between March 1997 and February 2006 were examined. Patients with dermatitis herpetiformis were not included. Results A total of 1402 patients were diagnosed with ABD during the study period. Pemphigus vulgaris (PV) was the most common ABD (81.2%), followed by bullous pemphigoid (BP) (11.6%), pemphigus foliaceus (PF) (4.4%), pemphigoid gestationis (0.7%), mucous membrane pemphigoid (0.7%), epidermolysis bullosa acquisita (0.5%), linear immunoglobulin A (IgA) disease (0.4%), paraneoplastic pemphigus (0.2%), IgA pemphigus (0.2%), and pemphigus erythematosus (0.1%). The mean age at diagnosis and male : female ratio were 43.4 years and 1 : 1.39, 42.2 years and 1 : 1.34, and 59.4 years and 1 : 1.36 for PV, BP, and PF, respectively. Conclusions Pemphigus vulgaris was the most frequent ABD according to our study; cases of PV outnumbered those of BP by a ratio of almost 8 : 1. This finding contrasts with those of studies conducted in Western European countries, in which BP predominates. There was a female predominance in most subtypes of ABD. Mean age at onset of PV and BP was lower than in Europe. In view of its large population of PV patients, Iran should be considered a suitable field for future clinical trials.     

Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study

Background Bullous pemphigoid (BP), pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune bullous diseases characterized by the presence of tissue‐bound and circulating autoantibodies directed against disease‐specific target antigens of the skin. Although rare, these diseases run a chronic course and are associated with significant morbidity and mortality. There are few prospective data on gender‐ and age‐specific incidence of these disorders. Objectives Our aims were: (i) to evaluate the incidence of BP and PV/PF in Swiss patients, as the primary endpoint; and (ii) to assess the profile of the patients, particularly for comorbidities and medications, as the secondary endpoint. Methods The protocol of the study was distributed to all dermatology clinics, immunopathology laboratories and practising dermatologists in Switzerland. All newly diagnosed cases of BP and pemphigus occurring between 1 January 2001 and 31 December 2002 were collected. In total, 168 patients (73 men and 95 women) with these autoimmune bullous diseases, with a diagnosis based on clinical, histological and immunopathological criteria, were finally included. Results BP showed a mean incidence of 12·1 new cases per million people per year. Its incidence increased significantly after the age of 70 years, with a maximal value after the age of 90 years. The female/male ratio was 1·3. The age‐standardized incidence of BP using the European population as reference was, however, lower, with 6·8 new cases per million people per year, reflecting the ageing of the Swiss population. In contrast, both PV and PF were less frequent. Their combined mean incidence was 0·6 new cases per million people per year. Conclusions This is the first comprehensive prospective study analysing the incidence of autoimmune bullous diseases in an entire country. Our patient cohort is large enough to establish BP as the most frequent autoimmune bullous disease. Its incidence rate appears higher compared with other previous studies, most likely because of the demographic characteristics of the Swiss population. Nevertheless, based on its potentially misleading presentations, it is possible that the real incidence rate of BP is still underestimated. Based on its significant incidence in the elderly population, BP should deserve more public health concern.     

Neonatal Autoimmune Blistering Disease: A Systematic Review

We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline, Embase, PubMed, Latin American and Caribbean Health Sciences Literature, and reference lists of identified articles. Inclusion criteria were articles published from 1946 to December 2014 in any language. Exclusion criteria were age greater than 4 weeks and no confirmed AIBD diagnosis. We identified 51 cases of neonatal AIBDs: 34 cases of pemphigus (31 pemphigus vulgaris [PV], 3 pemphigus foliaceus [PF]) and 17 cases of pemphigoid diseases (9 bullous pemphigoid [BP], 5 linear immunoglobulin A bullous dermatosis [LABD], 1 BP and LABD, 1 epidermolysis bullosa acquisita, 1 bullous systemic lupus erythematosus). Pemphigoid diseases had a higher male predominance (male:female ratio 4.6:1) than pemphigus (male:female ratio 1:1.06) (p = 0.004). Pemphigus had a higher proportion presenting at birth (79.4%) than pemphigoid diseases (29.4%) (p = 0.008). The most common sites involved were the trunk (63.0%), followed by the head and neck (60.9%). The mucosal membranes were involved in 32.6% of cases (27.6% in pemphigus, 41.6% in pemphigoid diseases). Only 33.3% used systemic therapy, and 75.5% achieved control within 3 weeks. Most PV, PF, and BP cases, but not LABDs, reported maternal disease. In pemphigus cases, 75.0% of mothers had active disease and 25.0% were in control. Pregnant women with PV, PF, and PG of any severity can passively transfer autoantibodies leading to neonatal AIBD. Pemphigoid diseases are more likely to present after birth and may be more male predominant. The presentation of LABDs may be different from that of all other AIBDs.     

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.     

Tetracycline and niacinamide control bullous pemphigoid but not pemphigus foliaceus when these conditions coexist

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.     

Erfolgreiche Therapie des Pemphigus foliaceus mit Rituximab

Treatment of autoimmune bullous diseases, especially of the pemphigus diseases, regularly requires the use of immunosuppressive drugs, often with insufficient clinical benefit but considerable side effects. A variety of autoimmune diseases (such as rheumatoid arthritis, lupus erythematosus, pemphigus vulgaris) have been successfully treated with rituximab, a chimeric monoclonal antibody against CD20, leading to a transient depletion of B cells. We report on three patients with pemphigus foliaceus who responded to rituximab after failing multiple other immunosuppressive therapies. We also examine the controversial issue of continuation therapy with rituximab in detail.     

Lesional Th17 cells and regulatory T cells in bullous pemphigoid

Th17 cells play crucial roles in the pathogenesis of autoimmune diseases. We previously reported that Th17 cells are recruited to the lesional skin in pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The aim of this study was to evaluate lesional Th17 cells and Treg cells in bullous pemphigoid (BP). Correlations between these cells and disease severity of BP were also evaluated. Immunohistochemical studies showed that both IL-17+ and Foxp3+ cells were present in higher numbers in BP lesions, compared with control skin. IL-17/CD4 ratio in BP was significantly higher than that in PF. Foxp3/CD4 ratio in BP was significantly less than that in either PV or PF. There were no obvious correlations between these cells and disease severity of BP. This study suggests that, compared with pemphigus, BP shows more Th17 cell-related inflammation and less Treg-related regulation.     

Oral cyclophosphamide for treatment of pemphigus vulgaris and foliaceus

BACKGROUND: Cyclophosphamide is an alkylating adjuvant used in refractory cases of pemphigus. OBJECTIVE: We sought to evaluate the effectiveness and safety of oral cyclophosphamide in the treatment of patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) with refractory disease. PATIENTS: We studied 23 patients with pemphigus (20 with PV; 3 with PF) who failed to achieve clinical remissions with the use of prednisone and antimetabolites. RESULTS: Complete remission was achieved in 17 patients with PV and 2 with PF. A total of 3 patients with PV failed therapy. A partial remission was achieved in 1 patient with PF. The treatment was administered for a median duration of 17 months with a follow-up period of 27 months. The median time to complete remission was 8.5 months. A total of 9 patients who were severely affected received concomitant plasma exchange. Adverse reactions included 5 cases of hematuria, 6 nonlife-threatening infections, and the development of transitional cell carcinoma of the bladder 15 years after discontinuation of cyclophosphamide in 1 patient. No death was associated with cyclophosphamide treatment. CONCLUSION: Oral cyclophosphamide is an effective adjuvant in the treatment of severe and refractory PV and PF, but requires close monitoring.     

Statistical observations of pemphigus at the Nara University Hospital from 1975 to 1989

From 1975 to 1989, 12 patients with pemphigus vulgaris (PV) and 19 with pemphigus foliaceus (PF) were treated with systemic or topical corticosteroids at the Nara University Hospital. All 12 patients with PV were treated with oral corticosteroids (initial dose of prednisolone: 15-70 mg/day) and 4 of 12 patients showed prolonged clinical remission (up to 9 years) without corticosteroids. Of 19 patients with PF, 16 patients were treated with oral corticosteroids (initial dose of prednisolone: 8-40 mg/day) and 3 patients were treated with only topical application of corticosteroids. In PF, 7 of 16 patients treated with systemic corticosteroids and all 3 patients treated with topical corticosteroids also showed prolonged clinical remission (up to 10 years). These observations suggest that most of the patients with pemphigus respond well to the treatment of relatively small or moderate dosage of corticosteroids.     

天疱疮或大疱性类天疱疮合并单纯疱疹病毒感染8例临床特征及治疗分析

目的回顾分析天疱疮/大疱性类天疱疮(BP)合并单纯疱疹病毒(HSV)感染的临床特征及治疗方法。方法回顾分析2016—2021年在武汉市第一医院住院治疗的天疱疮/BP合并HSV感染病例的临床特征及治疗和随访情况。结果 8例天疱疮/BP合并HSV感染患者中, 男2例, 女6例, 年龄(50.6 ± 8.3)岁, 包括5例寻常型天疱疮, 1例落叶型天疱疮, 2例BP。7例合并HSV-1感染, 1例合并HSV-2感染。8例均因天疱疮或BP接受系统糖皮质激素及免疫抑制剂治疗, 并对治疗抵抗入院, 其中7例表现为原发病灶加重或复发, 1例表现为全身皮损增加。HSV感染位于躯干4例, 口腔4例, 头皮3例, 面部2例。皮疹表现为不规则的糜烂面, 伴血痂, 部分为中央有脐凹的脓疱, 7例伴有皮疹处明显疼痛。发生HSV感染时, 6例天疱疮患者抗Dsg1抗体均下降, 5例寻常型天疱疮中4例抗Dsg3抗体下降;2例BP患者中1例抗BP180抗体降低, 1例升高。予足量足疗程抗病毒治疗(伐昔洛韦或更昔洛韦治疗7 ～ 14 d), 所有患者HSV感染均被控制, 自身免疫性大疱性皮肤病严重程度评分较抗病毒治疗前均...     

Rituximab in refractory autoimmune bullous diseases

Treatment of autoimmune blistering diseases consists of systemic glucocorticosteroids usually in combination with additional immunosuppressants such as azathioprine and mycophenolate mofetil or immunomodulators such as dapsone, antibiotics, intravenous immunoglobulins, and immunoadsorption. In some patients, these treatment regimens are not sufficient to control disease activity and/or lead to intolerable adverse events. Rituximab, originally developed for the treatment of non-Hodgkin's lymphoma, is an anti-CD20 humanized monoclonal antibody leading to transitory B-cell depletion. For this indication, rituximab is widely employed, and severe side-effects rarely observed. Subsequently, the B-cell-depleting effect of rituximab has been exploited successfully in various autoimmune disorders, including autoimmune blistering diseases. Here, we review the effect of rituximab in such diseases. To date, application of rituximab has been reported in 26 treatment-resistant patients with the vulgaris, foliaceus, and paraneoplastic variants of pemphigus as well as in bullous pemphigoid and epidermolysis bullosa acquisita. All but a single patient showed clinical improvement with reduction of lesion formation. In about a third, a clinical remission requiring further immunsuppressive medication was achieved, and in about a quarter, complete remission was induced. In addition, the mode of action and adverse events of rituximab as well as adjuvant immunosuppressive treatments, and the effect on levels of circulating autoantibodies in these patients are discussed.     

Adjuvant treatment of recalcitrant bullous pemphigoid with immunoadsorption

Elimination of pathogenic autoantibodies by immunoadsorption (IA) has been described as an effective adjuvant treatment in severe bullous autoimmune diseases, especially in pemphigus. There is much less experience in the treatment of bullous pemphigoid (BP). BP was diagnosed in a 62-year-old Caucasian woman presenting a pruritic rash with multiple tense blisters. Standard treatments with topical and oral corticosteroids, steroid-sparing agents including dapsone, azathioprine, mycophenolate mofetil (MMF) and intravenous immunoglobulins were ineffective or had to be discontinued due to adverse events. An immediate clinical response could be achieved by two treatment cycles of adjuvant protein A immunoadsorption (PA-IA) in addition to continued treatment with MMF (2 g/day) and prednisolone (1 mg/kg/day). Tolerance was excellent. Clinical improvement remained stable after discontinuation of IA and went along with sustained reduction of circulating autoantibodies. Our data demonstrate that PA-IA might be a safe and effective adjuvant treatment in severe and recalcitrant BP.     

Pemphigus vulgaris and pemphigus foliaceus: similar prognosis?

BACKGROUND: Pemphigus is a rare, life-threatening, acquired autoimmune bullous dermatosis. The prognosis of pemphigus foliaceus (PF) is usually regarded as more favourable than that of pemphigus vulgaris (PV). Our study aims to compare the clinical course of PV and PF in 37 patients. PATIENTS AND METHODS: We conducted a retrospective study over a period of eight years (1994-2001). The patients were referred during this period and were followed until December 2003. PF and PV were included based on clinical, histological and immunopathological criteria. RESULTS: In our study there was no significant difference between PF group and PV group concerning; age, sex, duration of the disease, presence of disseminated lesions, treatment, healing time, remission, relapse, complications, death and follows up duration. The survival graph showed no difference between the two groups for the first two relapses. There was a tendency to significance concerning an additional treatment and relapses frequency in the PF group. CONCLUSIONS: Few studies in the literature were interested in the evolution of the two forms of pemphigus. They showed that the two populations share the same clinical course; nevertheless they revealed the frequency of partial remission, failed treatment, relapses, necessity of high dose of corticosteroids, and difficulties of discontinuing treatment in PF. Our study, suggests that PF and PV may share the same clinical course.     

An evaluation of the usefulness of mycophenolate mofetil in pemphigus

BACKGROUND: 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.