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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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(CASE 2): A: erosions on face and scalp, after the third rituximabinfusion. B: healing of lesions on the face three weeks after thefourth rituximab infusion, increase of prednisone dose and introduction of MMF.C: face and scalp 18 months after the end of rituximabinfusions
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f03: (CASE 2): A: erosions on face and scalp, after the third rituximabinfusion. B: healing of lesions on the face three weeks after thefourth rituximab infusion, increase of prednisone dose and introduction of MMF.C: face and scalp 18 months after the end of rituximabinfusions

Mentions: Case 2 - Female, 46 years old, with hypertension and diabetes, both difficult tocontrol, presented bullous lesions and erosions, with purulent secretion, disseminatedon the scalp, face, trunk and limbs. Diagnosis of PV was confirmed histologically. Shewas hospitalized and submitted to treatment with antibiotic therapy, pulses ofmethylprednisolone IV and pulses of cyclophosphamide IV, with monthly intervals, besidesoral prednisone and azathioprine. She achieved almost complete remission of lesionsafter the second pulse of cyclophosphamide, with hospital discharge. After the fifthpulse she presented leucopenia and after the sixth pulse there was relapse of thedisease (Figure 2A). The patient was hospitalizedagain and rituximab infusions were administered with two-week intervals, maintainingoral prednisone and azathioprine. There was improvement in the trunk lesions, butworsening of the facial ones, even after the third infusion of rituximab (Figures 2B and 3A). As a consequence, together with the fourth infusion the prednisone dosewas increased and azathioprine replaced with MMF, with progressive improvement andhospital discharge after two months of hospitalization (Figure 3B). Since then the prednisone dose was progressively reduced and twoyears after hospital discharge, the patient remains with the disease under control withMMF 1g/day and prednisone 5mg/day (Figure 3C). Thetherapeutic steps, with drugs, doses, intervals and duration, are described in table 2.


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 2): A: erosions on face and scalp, after the third rituximabinfusion. B: healing of lesions on the face three weeks after thefourth rituximab infusion, increase of prednisone dose and introduction of MMF.C: face and scalp 18 months after the end of rituximabinfusions
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f03: (CASE 2): A: erosions on face and scalp, after the third rituximabinfusion. B: healing of lesions on the face three weeks after thefourth rituximab infusion, increase of prednisone dose and introduction of MMF.C: face and scalp 18 months after the end of rituximabinfusions
Mentions: Case 2 - Female, 46 years old, with hypertension and diabetes, both difficult tocontrol, presented bullous lesions and erosions, with purulent secretion, disseminatedon the scalp, face, trunk and limbs. Diagnosis of PV was confirmed histologically. Shewas hospitalized and submitted to treatment with antibiotic therapy, pulses ofmethylprednisolone IV and pulses of cyclophosphamide IV, with monthly intervals, besidesoral prednisone and azathioprine. She achieved almost complete remission of lesionsafter the second pulse of cyclophosphamide, with hospital discharge. After the fifthpulse she presented leucopenia and after the sixth pulse there was relapse of thedisease (Figure 2A). The patient was hospitalizedagain and rituximab infusions were administered with two-week intervals, maintainingoral prednisone and azathioprine. There was improvement in the trunk lesions, butworsening of the facial ones, even after the third infusion of rituximab (Figures 2B and 3A). As a consequence, together with the fourth infusion the prednisone dosewas increased and azathioprine replaced with MMF, with progressive improvement andhospital discharge after two months of hospitalization (Figure 3B). Since then the prednisone dose was progressively reduced and twoyears after hospital discharge, the patient remains with the disease under control withMMF 1g/day and prednisone 5mg/day (Figure 3C). Thetherapeutic steps, with drugs, doses, intervals and duration, are described in table 2.

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