利妥昔单抗:一种用于治疗寻常型天疱疮的抗CD20单克隆抗体

Background: Rituximab is an anti-CD20 chimeric antibody that selectively targets B lymphocytes. Recently, it has been reported to be beneficial in treating pemphigus vulgaris. Objective: Our aim was to review the English-language literature on the treatment of pemphigus vulgaris (PV) with rituximab and to determine its efficacy and influence on clinical outcome(s). Material and methods: A retrospective review of the literature on the use of rituximab in the treatment of PV was conducted. Seventeen patients in 10 reports were described and their data were reviewed. Results: The majority of patients received one course of rituximab along with conventional immunosuppressive therapy as concomitant therapy; 88%of the patients demonstrated improvement. More than half of the patients were followed up for more than 6 months after rituximab treatment; they appeared to be clinically disease free, but were still receiving conventional immunosuppressive therapy. Side effects in most patients were transient and infusion related. Serious infections occurred in 4 patients. One patient died. Limitations: The sample size of this study is small; there is no uniformity of data collection or measurement of key and critical indices, and follow-up was limited. Conclusion: Rituximab may be a promising agent in treatment of PV.

女性生殖道出现寻常型天疱疮

BACKGROUND: Involvement of the genital tract in women with pemphigus vulgaris has rarely been reported. We report 34 patients with pemphigus vulgaris who have involvement of the vulva, vagina, or both. METHODS: We reviewed the clinical data on 34 women treated at a tertiary dermatology referral center from July 1988 to June 2005. RESULTS: Of 34 patients, 21 had labial involvement, 3 had vaginal involvement, and 10 had both labial and vaginal involvement. The mean age of onset of pemphigus vulgaris was 49 years. All patients had pemphigus vulgaris involvement at multiple other sites, and all had other mucous membrane involvement, most commonly oral (97%). Recurrent episodes of genital pemphigus vulgaris were noted in 35%, with a mean of 6 recurrences (range 1-11). In all the patients the pemphigus vulgaris was controlled with systemic and local therapy. Long-term follow-up for a mean of 76 months (range 9-204) indicated that no long-term sequela occurred due to genital involvement. CONCLUSJON: Involvement of the female genital tract in pemphigus vulgaris is rare. Vulvar lesions occurmore commonly than vaginal lesions. With appropriate topical and systemic therapy, patients can have full recovery with no sequelae.

累及肛门的寻常型天疱疮

INTRODUCTION: Anal involvement in patients with pemphigus vulgaris has rarely been reported. We report 16 pemphigus vulgaris patients with anal involvement. METHODS: We retrospectively reviewed the clinical data on 16 patients treated at a tertiary dermatology referral center. RESULTS: Of 16 patients with anal involvement of pemphigus vulgaris, ten were female and six were male. The mean age of onset was 56 (range, 37-82) years. All patients had involvement of pemphigus vulgaris at multiple sites, including oral involvement. Recurrent episodes of anal pemphigus vulgaris were noted in nine (56 percent) patients, with a mean of 2.4 recurrences (range, 1-11). In all patients, pemphigus vulgaris was controlled with systemic and local therapy. Long-term follow-up for a mean of 53 (range, 4-188) months indicated that no long-term sequela occurred because of anal involvement. CONCLUSIONS: Anal involvement in pemphigus vulgaris is not very common and generally occurs in patients with severe disease. With appropriate topical and systemic therapy, patients have full recovery with no sequelae.