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Case Reports
. 2013 Jul-Aug;88(4):639-42.
doi: 10.1590/abd1806-4841.20131990.

Severe multi-resistant pemphigus vulgaris: prolonged remission with a single cycle of rituximab

Isabela Soubhia Corral  1 , Thais Helena Proença de Freitas, Renata Telles Rudge de Aquino, Daniella Abbruzzini S Koller, Maria Elisa Ruffolo Magliari, Helena Muller
Affiliations
Case Reports

Severe multi-resistant pemphigus vulgaris: prolonged remission with a single cycle of rituximab

Isabela Soubhia Corral et al. An Bras Dermatol. 2013 Jul-Aug.

Abstract
in English, Portuguese

Pemphigus vulgaris is an autoimmune bullous disease whose therapy is based on systemic corticosteroids, with or without immunosuppressants. Rituximab is a chimeric monoclonal antibody of the IgG class, directed at a specific CD20 B cell surface antigen, used in pemphigus vulgaris empirically since 2002, with success in 90% of the cases and long periods of remission. Male patient, 33 years old, diagnosed with pemphigus vulgaris, confirmed by histopathology and direct immunofluorescence. He was treated for seven months with numerous treatments, including immunosuppressive drugs, with an unsatisfactory response, until he had complete remission with the use of rituximab. During a 34-month follow-up period, the patient presented a slight clinical relapse, which was successfully controlled with prednisone in a daily dose of 120 mg, soon reduced to 20mg.

Pênfigo Vulgar é uma doença bolhosa auto-imune, cuja terapêutica é baseada em corticoesteróides sistêmicos, associados ou não a imunossupressores. Rituximabe é um anticorpo monoclonal quimérico da classe IgG direcionado a um antígeno CD20 de superfície celular específico da célula B, usado em pênfigo vulgar desde 2002, com sucesso em 90% e longos períodos de remissão. Paciente masculino, 33 anos, diagnóstico de pênfigo vulgar, confirmado por histopatologia e imunofluorescência direta. Durante 7 meses, recebeu inúmeros tratamentos com imunossupressores, apresentando resposta insatisfatória e progressão da doença, até que logo após a introdução de rituximabe teve completa remissão. Durante um acompanhamento de 34 meses, apresentou leve recidiva clínica, controlada com prednisona 120mg/dia, rapidamente reduzida e em uso atual de Prednisona 20mg/dia.

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Conflict of interest statement

Conflict of interest: None

Figures

FIGURE 1
FIGURE 1
01/05/2009 – Before rituximab
FIGURE 2
FIGURE 2
10/09/2009 – Kaposi varicelliform eruption
FIGURE 3
FIGURE 3
03/02/2010- 3 months after rituximab
FIGURE 4
FIGURE 4
20/07/2012 – 34 months after rituximab
See this image and copyright information in PMC

References

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