Refractory pemphigus vulgaris treated with rituximab and mycophenolate
                  mofetil

Biot, Stephanie Del Rio Navarrete; Franco, Joanna Pimenta de Araujo; Lima, Ricardo Barbosa; Pereira, Henrique Novo Costa; Marques, Luiz Paulo José; Martins, Carlos José

doi:10.1590/abd1806-4841.20143128

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Case Report • An. bras. dermatol. 89 (6) • Nov-Dec 2014 • https://doi.org/10.1590/abd1806-4841.20143128 copy

Refractory pemphigus vulgaris treated with rituximab and mycophenolate mofetil^* * Work carried out at the Hospital Universitário Gaffrée e Guinle, Universidade Federal do Estado do Rio de Janeiro (UNIRIO) - Rio de Janeiro (RJ), Brasil.

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The main treatment for pemphigus vulgaris are systemic corticosteroids and immunosuppressive agents, but due to adverse reactions and therapeutic failure, new drugs such as rituximab and mycophenolate mofetil have been used. In this case report are described two cases of severe pemphigus vulgaris refractory to various treatments, with resolution after use of rituximab and mycophenolate mofetil, associated with corticosteroids. A higher-than-usual dose of rituximab was employed, without the occurrence of serious adverse reactions. Mycophenolate mofetil was added as adjunctive therapy due to lack of response to azathioprine.

Antibodies, monoclonal; Immunosuppressive agents; Pemphigus

Phases	Medications	Duration
1st	Pulse therapy with MPD lg/day IV, for 3 days, and 6 pulses CPP 500mg/m2 IV, with monthly intervals and PDN 30mg/day and oral AZA 150mg/day between pulses. Hospital discharge after second pulse of CPP. Following pulses in a day-hospital schedule.	22 weeks
2nd	New hospitalization for administration of RTX, 4 infusions of lg, with 15-day interval, continuing PDN 30mg/day and AZA 150mg/day up to the 4th infusion. With the 4th infusion, PDN was increased to 80mg/day and AZA was replaced by MMF 1.5g/day. Hospital discharge after 2 months.	9 weeks
3rd	Outpatient clinic treatment with MMF 1.5g/day and progressive decrease of PDN dose. Two years after the last RTX infusion, the disease remains controlled with MMF 1g/day and PDN 5mg/day.	24 weeks

Sociedade Brasileira de Dermatologia Av. Rio Branco, 39 18. and., 20090-003 Rio de Janeiro RJ, Tel./Fax: +55 21 2253-6747 - Rio de Janeiro - RJ - Brazil
E-mail: revista@sbd.org.br

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[1] MAILING ADDRESS: Stephanie Del Rio Navarrete Biot, Rua Mariz e Barros, 775 - Tijuca, 20270-004 - Rio de Janeiro - RJ, Brazil. E-mail: tupibiot@hotmail.com

Phases	Medications	Period
1st	2 cycles of pulse therapy with MPD 1g IV, for 3 days, with weekly interval and PDN 60 mg/day, between pulses.	2 weeks
2nd	3 cycles of infusions of IVIG 2g/kg/cycle, divided in 4 days, with intervals of 2 to 3 weeks, PDN 120mg/day and AZA 150mg/day.	9 weeks
3rd	4 pulses of CPP 500mg/m² IV, with interval of 3 weeks between pulses and PDN 120mg/day.	9 weeks
4th	3 sessions of PPH with 1 pulse of CPP on fourth day, and PDN 120mg/day	2 weeks
5th	RTX, 2 cycles of 4 weekly infusions of 375mg/m², with 4-week intervals between cycles, PDN 120mg/day and MMF 2g/day. Hospital discharge after 8 months.	12 weeks
6th	Outpatient clinic treatment with MMF 2g/day and progressive reduction of PDN dose for 1 year, until suspension, when relapse of lesions occurred, refractory to reintroduction of PDN 80mg/day.	12 weeks
7th	New hospitalization for administration of RTX, 1 cycle of two infusions of 1g, with a two-week interval, PDN 80mg/day and MMF 2.5g/day. Hospital discharge after 4 weeks.	4 weeks
8th	Outpatient clinic treatment with progressive reduction of PDN dose. One year after the last RTX infusion, the disease remains controlled with MMF 2.5mg and PDN 10mg on alternate days.	12 weeks

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Refractory pemphigus vulgaris treated with rituximab and mycophenolate mofetil* * Work carried out at the Hospital Universitário Gaffrée e Guinle, Universidade Federal do Estado do Rio de Janeiro (UNIRIO) - Rio de Janeiro (RJ), Brasil.

Refractory pemphigus vulgaris treated with rituximab and mycophenolate mofetil^* * Work carried out at the Hospital Universitário Gaffrée e Guinle, Universidade Federal do Estado do Rio de Janeiro (UNIRIO) - Rio de Janeiro (RJ), Brasil.