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

Corral IS, Freitas TH, Aquino RT, Koller DA, Magliari ME, Muller H - An Bras Dermatol (2013 Jul-Aug)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Dermatology Clinic, Santa Casa de Misericórdia de São Paulo, São Paulo(SP), Brazil.

ABSTRACT
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.

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03/02/2010- 3 months after rituximab
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f03: 03/02/2010- 3 months after rituximab

Mentions: The persisting lack of clinical response resulted in the use of cyclophosphamide pulsetherapy at 250mg/day, associated to dexamethasone 100 mg/day for 3 days. As the patientwas still unresponsive, weekly intravenous treatment with rituximab 375mg/m2 (4 cycles)was started. The patient presented with improvement of the cutaneous lesions after thefirst cycle and a complete remission in 20 days, being discharged with a prescription ofprednisone 2 mg/Kg/day and cyclophosphamide 50 mg/day (Figure 3). These medications were progressively withdrawn and the patient wasasymptomatic for 23 months using only low dosage corticoids (5mg/day). After this periodthere was a slight clinical relapse, which was controlled with prednisone 120 mg/day,resulting in rapid regression of the disease. Presently, after 34 months of follow-up,the patient is being treated only with prednisone 20 mg/day (Figure 4).


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

Corral IS, Freitas TH, Aquino RT, Koller DA, Magliari ME, Muller H - An Bras Dermatol (2013 Jul-Aug)

03/02/2010- 3 months after rituximab
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3760947&req=5

f03: 03/02/2010- 3 months after rituximab
Mentions: The persisting lack of clinical response resulted in the use of cyclophosphamide pulsetherapy at 250mg/day, associated to dexamethasone 100 mg/day for 3 days. As the patientwas still unresponsive, weekly intravenous treatment with rituximab 375mg/m2 (4 cycles)was started. The patient presented with improvement of the cutaneous lesions after thefirst cycle and a complete remission in 20 days, being discharged with a prescription ofprednisone 2 mg/Kg/day and cyclophosphamide 50 mg/day (Figure 3). These medications were progressively withdrawn and the patient wasasymptomatic for 23 months using only low dosage corticoids (5mg/day). After this periodthere was a slight clinical relapse, which was controlled with prednisone 120 mg/day,resulting in rapid regression of the disease. Presently, after 34 months of follow-up,the patient is being treated only with prednisone 20 mg/day (Figure 4).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Dermatology Clinic, Santa Casa de Misericórdia de São Paulo, São Paulo(SP), Brazil.

ABSTRACT
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.

Show MeSH
Related in: MedlinePlus