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Refractory pemphigus vulgaris treated with rituximab and mycophenolate mofetil.

Biot Sdel R, Franco JP, Lima RB, Pereira HN, Marques LP, Martins CJ - An Bras Dermatol (2014 Nov-Dec)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Universidade Federal do Estado do Rio de Janeiro (UNIRIO), Rio de Janeiro, RJ, Brazil.

ABSTRACT
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.

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Related in: MedlinePlus

(CASE 1): A: extensive erosion of lesions on the trunk and limbs, inthe first phase of exacerbation of the disease before beginning treatment withintravenous immunoglobulin. B: dorsolumbar region afterplasmapheresis, before beginning treatment with rituximab. C:dorsolumbar region after the end of second cycle of rituximab, three months afterthe first infusion
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f01: (CASE 1): A: extensive erosion of lesions on the trunk and limbs, inthe first phase of exacerbation of the disease before beginning treatment withintravenous immunoglobulin. B: dorsolumbar region afterplasmapheresis, before beginning treatment with rituximab. C:dorsolumbar region after the end of second cycle of rituximab, three months afterthe first infusion

Mentions: Case 1 - Male, 47 years old, with diagnosis of PV and using prednisone 120mg/day for onemonth. At the examination he presented eroded and crusted lesions disseminated on theskin and erosions in the oral mucosa. Diagnosis of PV was histologically confirmed.Treatment was begun with pulses of methylprednisolone IV and oral prednisone betweenpulses. The patient progressed rapidly with severe worsening and infusions ofintravenous immunoglobulin combined with prednisone and oral azathioprine wereintroduced (Figure 1A). After a period ofimprovement new lesions appeared and pulses of cyclophosphamide IV were administered,continuing with oral prednisone. Again there was improvement but after the fourth pulsea severe relapse occurred. Plasmapheresis was performed as well as a new pulse ofcyclophosphamide with good response, but after seven days there was onset of new lesions(Figure 1B). At this point, treatment withrituximab was initiated. Prednisone was maintained and MMF was added. After the secondcycle there was complete resolution of the clinical picture with hospital discharge,main-taining prednisone and MMF (Figure 1C). Thepatient was hospitalized for eight months and presented severe complications likeinfections and sepsis, before the initiation of rituximab, making use of several schemesof antibiotic therapy. During the outpatient clinic follow-up the MMF dose wasmaintained and the dose of prednisone was progressively reduced in the course of oneyear, until suspension. At this point, oral lesions appeared which persisted even afterincrease of prednisone. A new infusion of rituximab was started and the dose of MMF wasincreased, with resolution of lesions. One year after the last cycle of rituximab, thepatient remains with the disease controlled with MMF 2.5g/day and prednisone 10mg onalternate days. The therapeutic steps with drugs, doses, intervals and duration, aredescribed in table 1.


Refractory pemphigus vulgaris treated with rituximab and mycophenolate mofetil.

Biot Sdel R, Franco JP, Lima RB, Pereira HN, Marques LP, Martins CJ - An Bras Dermatol (2014 Nov-Dec)

(CASE 1): A: extensive erosion of lesions on the trunk and limbs, inthe first phase of exacerbation of the disease before beginning treatment withintravenous immunoglobulin. B: dorsolumbar region afterplasmapheresis, before beginning treatment with rituximab. C:dorsolumbar region after the end of second cycle of rituximab, three months afterthe first infusion
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4230671&req=5

f01: (CASE 1): A: extensive erosion of lesions on the trunk and limbs, inthe first phase of exacerbation of the disease before beginning treatment withintravenous immunoglobulin. B: dorsolumbar region afterplasmapheresis, before beginning treatment with rituximab. C:dorsolumbar region after the end of second cycle of rituximab, three months afterthe first infusion
Mentions: Case 1 - Male, 47 years old, with diagnosis of PV and using prednisone 120mg/day for onemonth. At the examination he presented eroded and crusted lesions disseminated on theskin and erosions in the oral mucosa. Diagnosis of PV was histologically confirmed.Treatment was begun with pulses of methylprednisolone IV and oral prednisone betweenpulses. The patient progressed rapidly with severe worsening and infusions ofintravenous immunoglobulin combined with prednisone and oral azathioprine wereintroduced (Figure 1A). After a period ofimprovement new lesions appeared and pulses of cyclophosphamide IV were administered,continuing with oral prednisone. Again there was improvement but after the fourth pulsea severe relapse occurred. Plasmapheresis was performed as well as a new pulse ofcyclophosphamide with good response, but after seven days there was onset of new lesions(Figure 1B). At this point, treatment withrituximab was initiated. Prednisone was maintained and MMF was added. After the secondcycle there was complete resolution of the clinical picture with hospital discharge,main-taining prednisone and MMF (Figure 1C). Thepatient was hospitalized for eight months and presented severe complications likeinfections and sepsis, before the initiation of rituximab, making use of several schemesof antibiotic therapy. During the outpatient clinic follow-up the MMF dose wasmaintained and the dose of prednisone was progressively reduced in the course of oneyear, until suspension. At this point, oral lesions appeared which persisted even afterincrease of prednisone. A new infusion of rituximab was started and the dose of MMF wasincreased, with resolution of lesions. One year after the last cycle of rituximab, thepatient remains with the disease controlled with MMF 2.5g/day and prednisone 10mg onalternate days. The therapeutic steps with drugs, doses, intervals and duration, aredescribed in table 1.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Universidade Federal do Estado do Rio de Janeiro (UNIRIO), Rio de Janeiro, RJ, Brazil.

ABSTRACT
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.

Show MeSH
Related in: MedlinePlus