Severe Multi-Resistant Pemphigus vulgaris: prolonged remission with a\nsingle cycle of Rituximab
Male patient, 33 years-old, without any morbid antecedents of notice, was admitted to\nthe hospital with a history of painful bullous lesions on the scalp and oral cavity\nsince two months before, with subsequent dissemination to the entire body. Physical\nexamination showed flaccid blisters, mostly with serous content, besides exulcerated\nlesions covered in honey-colored crusts. () The diagnostic hypothesis was pemphigus vulgaris, later confirmed by skin\nbiopsies and direct immunofluorescence. Treatment with prednisone 1mg/Kg/day and\nantibiotics to address the secondary infection was then started. The patient was\nreleased 15 days later, with a slightly improved clinical condition.\nHe returned 10 days later, due to the appearance of new blisters, worsening of the pain\nand odynophagia. The dose of corticoid was increased to 1.5mg/Kg/day, and both sulfone\n100 mg/day and systemic antibiotics were added to the treatment. As the patient's\nclinical condition rapidly deteriorated, sulfone was replaced by azathioprine 150 mg/day\nand the dose of prednisone was once again increased to 2mg/Kg/day. Despite these\nactions, there was a decline in the patient's general condition, and a worsening of the\ncutaneous lesions, accompanied by the development of thrombocytopenia secondary to the\nuse of azathioprine. In a period of 20 days, the patient had almost 100% of his body\nsurface stricken by the disease, including all mucosae (oral, nasal, ocular, penile,\nanal) and the periungual regions of all fingers and toes. He was then submitted to\nintravenous methylprednisolone pulse therapy in a dose of 1g/day for 5 days. A week\nafter the pulse therapy, new lesions were still appearing, leading to the introduction\nof five cycles of intravenous immunoglobulin at 400mg/Kg/per cycle. After 75 days of\nhospitalization, the patient was discharged with an improved clinical condition.\nOne month after being discharged, the patient had a clinical relapse, with the\nappearance of new bullous lesions despite the daily use of prednisone 2mg/Kg. A new\ncycle of intravenous immunoglobulin was performed, followed by a second pulse therapy\nwith methylprednisolone and the addition of mycophenolate mofetil 3g/day for 30 days.\nHowever, mycophenolate mofetil was suspended due to Kaposi varicelliform eruptions,\nwhich were treated with intravenous acyclovir ().\nThe persisting lack of clinical response resulted in the use of cyclophosphamide pulse\ntherapy at 250mg/day, associated to dexamethasone 100 mg/day for 3 days. As the patient\nwas still unresponsive, weekly intravenous treatment with rituximab 375mg/m2 (4 cycles)\nwas started. The patient presented with improvement of the cutaneous lesions after the\nfirst cycle and a complete remission in 20 days, being discharged with a prescription of\nprednisone 2 mg/Kg/day and cyclophosphamide 50 mg/day (). These medications were progressively withdrawn and the patient was\nasymptomatic for 23 months using only low dosage corticoids (5mg/day). After this period\nthere was a slight clinical relapse, which was controlled with prednisone 120 mg/day,\nresulting in rapid regression of the disease. Presently, after 34 months of follow-up,\nthe patient is being treated only with prednisone 20 mg/day ().