About 24 80% patients had severe skin and mucosal involvement 30% body. As a result of the relative rarity of pv, published randomized controlled trials rcts are limited, which makes it difficult to evaluate the efficacy of different treatment regimens. Pemphigus vulgaris is an uncommon autoimmune blistering dermatosis characterized by painful mucocutaneous erosions. Black st johns institute of dermatology, st thomas hospital, london, se1 7eh u. In three cases of pemphigus vulgaris and one case of pemphigus foliaceus, dexamethasone and cyclophosphamide pulse therapy associated with prednisone helped the lesions to heal more rapidly. The administration of suprapharmacologic doses of drugs in an intermittent manner is known as pulse therapy. Oral pemphigus without skin lesions treated with pulse steroid therapy nitya kala 1, jayakumar manjeu 1, neil dominic 2, srinivasan poovan kirubanidhi kennedy babu 1 1 department of periodontology, mahatma gandhi postgraduate institute of dental sciences, puducherry, india 2 department of dentistry, indira gandhi medical college and research institute, puducherry, india. Pemphigus vulgaris 1,2,3,10,11 patient has a diagnosis of pemphigus vulgaris as determined by the following. Successful removal of pathogenic autoantibodies in pemphigus by immunoadsorption with a tryptophanlinked polyvinylalcohol adsorber. The aim of this study is to evaluate the efficacy and safety of steroid pulse therapy in treating pv. Patients who had received previous steroid pulse therapy for any reasons or had major organ dysfunction were excluded from the study.
However, there is a paucity of data about the efficacy and safety of methylprednisolone, despite its frequent administration. Methylprednisolone pulse therapy plus adjuvant therapy for. Various studies have proven the efficacy of pulse therapy along with reduced side effects of conventional. We recently reported that mizoribine is effective for pemphigus vulgaris based on 11 patients. Pulse intravenous cyclophosphamide therapy in pemphigus. Neither treatment however prevented the recurrence of the disease. Fifty patients with pemphigus 45 pemphigus vulgaris, 5 pemphigus foliaceus were treated with dexamethasonecyclophosphamide pulse therapy. In pemphigus, pulse therapy refers to intravenous iv infusion of high doses of steroids for. Guidelines guidelines for the management of pemphigus vulgaris. Synchronization of plasmapheresis and pulse cyclophosphamide therapy in pemphigus vulgaris. Dcp therapy was started on 46 patients and dexamethasone pulse therapy was started in 5 females of reproductive age group who were yet to.
Pemphigus vulgaris is a potentially fatal disease in spite of a variety of treatment modalities available. A case of acute pemphigus vulgaris relapses associated. Introduction the treatment of oral mucosal disorders must be based on an early and correct diagnosis. It is generally agreed that patients with only limited. Desmoglein 1 is present in cns neurons and in all epithelial cells, and an immunologic crossreaction between epithelial and cns isoforms has been suggested. If you have an autoimmune disease, your immune system mistakenly attacks your. Results a total of 51 cases diagnosed with pemphigus were included in the study, of which 21 were males and 30 were females.
Cyclophosphamide pulse therapy for pemphigus, international journal of dermatology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. It can be a lifethreatening condition if left untreated. A 16yearold girl showed oral and cutaneous lesions suggestive of pemphigus. Dexamethasone cyclophosphamide pulse dcp therapy in. Prospective study of pulse therapy in childhood pemphigus. Pulse versus oral methylprednisolone therapy in pemphigus. Steroid pulse therapy has shown satisfactory efficacy and safety in treating pemphigus vulgaris pv. Between 1995 and 2001, we treated 41 patients of pemphigus with modified pulse therapy. Sir, pemphigus vulgaris is a potentially fatal disease in spite of a variety of treatment modalities available.
Intravenous pulse cyclophosphamide, which may be more efficacious and less toxic than oral immunosuppressives, has been used successfully in the treatment of pemphigus. In this study, five cases of pemphigus vulgaris and two cases of pemphigus foliaceus were treated with cyclophosphamide pulse therapy associated with prednisone, resulting in the need for a smaller maintenance dose of prednisone. The relapse rate seen in pemphigus vulgaris and pemphigus foliaceus were 21. Plasmapheresis and pulse cyclophosphamide therapy in. The aim of the study was to assess the outcome of pulse therapy in pemphigus patients treated over a period of 10 years. To see the efficacy of cyclophosphamide pulse therapy in pemphigus vulgaris. These modifications included certain changes in the dcp therapy protocol and substitution of cyclophosphamide with either azathioprine or methotrexate in a few patients. Many open studies differing in design, criteria, regimen, and concomitant drugs evaluated cs pulse therapy in pemphigus. In pemphigus, pulse therapy refers to intravenous iv infusion of high doses of steroids for one or more days for quicker, better efficacy, and to decrease the side effects of longterm steroids.
The objective of this paper is to highlight various parameters of dcp therapy. Guidelines guidelines for the management of pemphigus vulgaris k. Pemphigus vulgaris pv is a chronic, mucocutaneous, autoimmune bullous disease. Pulse therapy has been tried in pemphigus in india and other countries with variable results. Oral pemphigus without skin lesions treated with pulse. Patients were divided into two groups of intravenous steroid pulse therapy study group and conventional oral steroid therapy control group. Patients with pemphigus treated with pulse therapy from 2006 to 2016 were taken into the study. Aug 10, 2019 steroid pulse therapy has shown satisfactory efficacy and safety in treating pemphigus vulgaris pv. May 15, 2017 therapy is aimed at reducing antibody synthesis. Guidelines guidelines for the management of pemphigus. Pulse therapy appears to be successful in the treatment of pemphigus vulgaris. Pemphigus vulgaris merck manuals professional edition.
The pulse consisted of 6 mg dexamethasone dissolved in 5% dextrose given in a drip over a period of 12 hours on 3 consecutive days. Pemphigus vulgaris pv and mucous membrane pemphigoid mmp are among the diseases that pose the greatest diagnostic difficulties. A study on effectiveness of dexamethasonecyclophosphamide. However, pasricha and gupta 1 introduced the dexamethasonecyclophosphamide pulse dcp therapy in 1984, 1, 2 and thereafter, plenty of literature on the management of pemphigus in india is. The efficacy of cyclophosphamide pulse therapy was evaluated in pemphigus vulgaris 2 males and 4 females aged 34 to 47 years and in a 56yearold male with pemphigus foliaceus. Pemphigus vulgaris, corticosteroids, pulse therapy, cyclophosphamide, azathioprine. Dexamethasone cyclophosphamide pulse therapy commonly known as dcp therapy was introduced by pasricha et al in 1982 in india for treatment of pemphigus vulgaris. Two authors performed both the screening and selection of studies independently in order to ensure impartiality when selecting the studies. Pemphigus vulgaris and pemphigus foliaceus are autoimmune blistering diseases that may result in significant morbidity and death. Further studies on pemphigus patients treated with dexamethasonecyclophosphamide pulse therapy for pemphigus. Pulse therapy means administering a drug at suprapharmacological doses over a short period and then withdrawing it completely till next dose.
Traditional firstline treatment for pemphigus consists of systemic immunosuppression with systemic glucocorticoids with or without adjuvant immunosuppressive medications. In this study, five cases of pemphigus vulgaris and two cases of pemphigus foliaceus were treated with cyclophosphamide pulse therapy associated with prednisone, resulting in the need for a. During the induction phase of treatment, the study. Since then its use is extensively studied for long term remission and side effects in pemphigus vulgaris and other immunebullous disorders.
These modifications included certain changes in the dcp therapy protocol and substitution of. Apart from pemphigus disorders, dcp therapy was also found to be effective in pemphigoid disorders, systemic sclerosis, systemic lupus erythematous, etc. Comparison of cost of conventional immunosuppressive therapy cist with ivig therapy in patients with mucous membrane pemphigoid mmp, ocular cicatricial pemphigoid ocp, bullous pemphigoid bp and pemphigus vulgaris pv. However, even though there is enough evidence about its efficacy and methodology, there continue to be doubts. Efficacy and safety of dexamethasone cyclophosphamide pulse therapy in the treatment of pemphigus an open randomized controlled study author links open overlay panel ramji gupta a sachi gupta a anil gupta b. Pdf dexamethasonecyclophosphamide pulse therapy in. Pdf pemphigus is an autoimmune skin disease that can present in a variety of forms and can prove challenging to manage and treat. The efficacy of cyclophosphamide pulse therapy was evaluated in pemphigus vulgaris 2 males and 4 females aged 34 to 47 years and in a 56yearold male. If administered properly, dexamethasone cyclophosphamide pulse dcp therapy has the potential to effect lifelong recovery from pemphigus. A yearold child is described who presented with generalized pemphigus vulgaris associated with extraordinarily high titers of circulating autoantibodies against the pemphigus antigen. Dcp was introduced in the management of pemphigus vulgaris. Since then its use is extensively studied for long term remission and side effects in pemphigus vulgaris.
Efficacy and safety of dexamethasone cyclophosphamide pulse. Dcp therapy was started on 46 patients and dexamethasone pulse therapy. Pulse therapy for the treatment of pemphigus has been in vogue for. Pemphigus vulgaris can be present in different forms like oral and topical. Dexamethasone cyclophosphamide pulse therapy in pemphigus. The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events. Cyclophosphamide pulse therapy for pemphigus scielo.
However, even though there is enough evidence about its efficacy and methodology, there continue to be doubts and questions regarding the rationale of use of high dose intravenous steroids and steroidsparing immunosuppressants. Progress of pemphigus antibody pab titer in patient treated by massivevolume plasmaphereses followed by pulse cyclophosphamide therapy. Plasmapheresis can be a useful alternative or adjunctive intervention in pemphigus vulgaris that is not responding to conventional therapy. Pemphigus vulgaris is an autoimmune skin disorder with development of blisters in the skin and mucosa, and it can be a lifethreatening disease if not treated. Figure 1 and figure 2 demonstrate improvement in a patient with pemphigus vulgaris from our series. It is an autoimmune disease characterized by production of pathogenic autoantibodies directed against desmosome proteins of keratinocytes in the epidermis 1. Pdf p classabstractin pemphigus vulgaris, pulse therapy is a promising modality to achieve better therapeutic response and to minimize. These guidelines for management of pemphigus vulgaris have been prepared for dermatologists on. Here, we report the outcome of dexamethasone only pulse dop therapy in 12 pediatric patients with pemphigus vulgaris pv in terms of remission, side effects, and relapse.
Relapse introduction pemphigus is a rare blistering disease that affects the skin and mucous membranes. The place of pv among autoimmune bullous dermatoses is well established. Guidelines for the management of pemphigus vulgaris british. Fifty patients with pemphigus 45 pemphigus vulgaris, 5 pemphigus foliaceus were treated with dexameth. Systemic corticosteroids remain the gold standard treatment for pemphigus vulgaris. Dexamethasonecyclophosphamide pulse therapy in pemphigus. Pemphigus vulgaris is a rare autoimmune disease that causes painful blistering on the skin and mucous membranes.
A number of recent reports showed that intravenous highdose steroid pulse therapy such as methylprednisolone or dexamethasone is an effective method for the control of pemphigus vulgaris. Introduction pemphigus vulgaris pv is a chronic autoimmune blistering disease of skin and mucous membrane. The introduction of dexamethasonecyclophosphamide pulse dcp therapy for the pemphigus group of disorders by pasricha et al. The modifications of pulse therapy and dexamethasone. Curative effect of dexamethasonecyclophosphamide pulse therapy for the treatment of pemphigus vulgaris. It affects mainly middleaged adults, and juvenile cases are rare. Out of 30 patients, 28 was pemphigus vulgaris and 2 was pemphigus foliaceus. Pemphigus vulgaris, foliaceus, or both may coexist with certain central nervous system cns disorders, especially dementia, epilepsy, and parkinson disease. Pemphigus vulgaris pv is a chronic, autoimmune, vesiculobullous disease. Pulse versus oral methylprednisolone therapy in pemphigus vulgaris 2 archives of iranian medicine, volume 10, number 1, january 2007 therapy. Pemphigus vulgaris is an uncommon mucocutaneous disease caused by autoantibodies against desmosomal antigens.
Pulse therapy is recommended as an adjunct to the total management plan of severely affected patients with pemphigus vulgaris. A 48yearold chinese man first presented with mucocutaneous erosions 2 years ago, and a diagnosis of pemphigus vulgaris. The main objective in the treatment of pemphigus vulgaris. Pulse therapy for the treatment of pemphigus has been in vogue for several years and is administered by many dermatologists across the world. The recent report by pasricha et al, of successful treatment with dcp therapy of pemphigus patients is very promising and encouraged us to undertake this study. The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents. In three cases of pemphigus vulgaris and one case of pemphigus foliaceus, dexamethasone and cyclophosphamide pulse therapy associated with prednisone. Mortality rate due to pemphigus vulgaris can be decreased by using systemic corticosteroids, but the present treatment options are limited due to the toxicity profiles of drugs available for treating pemphigus vulgaris. The addition of cyclophosphamide may prevent the disease from recurring. The treatment of pemphigus has been a long standing. Accepted for publication 15 may 2003 summary these guidelines for management of pemphigus vulgaris have been prepared for dermatologists on.
In three cases of pemphigus vulgaris and one case of pemphigus foliaceus, dexamethasone. In three cases of pemphigus vulgaris and one case of pemphigus. Evaluation of cyclophosphamide pulse therapy as an adjuvant to oral corticosteroid in the management of pemphigus vulgaris. Introduction pemphigus vulgaris pv is a chronic autoimmune blistering disease of skin and. Pdf managing the challenging cases of pemphigus vulgaris with. Longlasting and complete remission was obtained in a 48yearold patient with refractory pemphigus vulgaris by an experimental treatment protocol that tries to synchronize plasmapheresis with subsequent pulse cyclophosphamide therapy. Pulse versus oral methylprednisolone therapy in pemphigus vulgaris. Pemphigus vulgaris is a potentially fatal, chronic, autoimmune mucocutaneous bullous disease, precipitated by igg autoantibodies binding to desmoglein 3 dsg3 and desmoglein 1 dsg1.
624 199 1057 961 900 55 210 412 690 1164 6 879 1440 802 966 354 12 484 654 1445 1212 1171 302 530 138 353 386 1502 1454 27 262 561 189 1124 1124 842 501