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      Dexamethasone-cyclophosphamide pulse therapy outcomes comparing pemphigus vulgaris and pemphigus foliaceus groups in a Brazilian cohort study

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          Abstract

          Background

          Dexamethasone-cyclophosphamide pulse (DCP) and dexamethasone pulse (DP) have been successfully used to treat pemphigus, but DCP/DP outcomes comparing pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are scarce.

          Objective

          To compare DCP/DP outcomes in a Brazilian cohort of PV and PF patients according to demographic and clinical data.

          Methods

          Retrospective analytical cohort study, reviewing medical charts of PV and PF patients (for DCP/DP Phases I‒IV consult Pasricha et al.16‒18).

          Results

          37 PV and 41 PF patients non responsive to usual treatments were included similarly for DCP or DP therapy. Disease duration was longer among PF before DCP/DP prescription (p < 0.001); PF required a higher number of monthly pulses to acquire remission in Phase I (median 10 and 6 pulses, respectively; p = 0.005). DCP/DP outcomes were similar in both groups: remission in 37.8% of PV and 34.1% of PF after completed DCP/DP cycles following a median of 13 months (1–56 months follow-up); failure occurred in 13.5% of PV and 14.6% of PF in Phase I; relapse in 13.5% of PV and 12.2% of PF, and dropout in 27% of PV and 24.4% of PF in Phases II to IV. Mild side effects were documented.

          Study limitations

          The severity of PV and PF disease was not assessed by score indexes.

          Conclusions

          PV and PF patients presented similar DCP/DP outcomes. DCP/DP should be initiated earlier in PF patients due to the longer duration of their disease in order to decrease the number of pulses and the duration of Phase I to acquire remission.

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          Most cited references33

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          Incidence and prevention of bladder toxicity from cyclophosphamide in the treatment of rheumatic diseases: a data-driven review.

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            Differential HLA class I and class II associations in pemphigus foliaceus and pemphigus vulgaris patients from a prevalent Southeastern Brazilian region

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              Pemphigus in India.

              Pemphigus is a chronic epidermal immunobullous disease with potentially fatal outcome. The journey of literature on pemphigus in India has come a long way in last five decades. Pemphigus in Indian patients has unique genetic, clinical, and epidemiological differences from those in the Western countries. Corticosteroids remain the mainstay of treatment for pemphigus. Dexamethasone-cyclophosphamide pulse therapy has revolutionized the management of pemphigus in India and abroad for nearly 3 decades now. Corticosteroid-based treatment, along with adjuvants, has significantly brought down the high mortality rates that had been observed in precorticosteroid era. Present day research is largely based on elucidating the pathogenesis beyond the antidesmoglein antibodies, and newer diagnostic and treatment approaches. In this article, we review various aspects of literature on pemphigus in India, on Indians abroad, or literature from other countries that are considered relevant to the topic.
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                Author and article information

                Contributors
                Journal
                An Bras Dermatol
                An Bras Dermatol
                Anais Brasileiros de Dermatologia
                Sociedade Brasileira de Dermatologia
                0365-0596
                1806-4841
                22 June 2023
                Nov-Dec 2023
                22 June 2023
                : 98
                : 6
                : 774-780
                Affiliations
                [a ]Department of Medical Clinics, Hospital Universitário, Universidade de São Paulo, Ribeirão Preto, SP, Brazil
                [b ]Department of Medicine, Universidade Federal do Delta do Parnaíba, Parnaíba, PI, Brazil
                [c ]Department of Medical Clinics, Universidade de São Paulo, Ribeirão Preto, SP, Brazil
                Author notes
                [* ]Corresponding author. amfrosel@ 123456fmrp.usp.br
                Article
                S0365-0596(23)00135-6
                10.1016/j.abd.2022.11.005
                10589458
                37355353
                d520a2ea-2b59-4064-9cf0-963426c05551
                © 2023 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U.

                This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

                History
                : 13 September 2022
                : 10 November 2022
                Categories
                Original Article

                corticoids,cyclophosphamide,pemphigus,pulse therapy, drug

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