No abstract

Our goal was to determine, retrospectively, the occurrence of the symptoms of Behçet's disease in chronological order and the course of the disease. Additionally, probable factors affecting the clinical severity were investigated. A total of 368 patients (171 females and 197 males; aged 41.11 ± 10.9 years) were included in this retrospective cohort study. The chronological order of the clinical manifestations was recorded. Patients were also assessed for clinical severity score. Oral ulcer was the most common manifestation (100 %) followed by genital ulcer (89.4 %), papulopustular lesions (75 %) and articular involvement (60.1 %). Oral ulcer was the most common onset manifestation (66.8 %) followed by genital ulcer (4.9 %), erythema nodosum (3.3 %) and ocular involvement (1.4 %). The duration between the onset symptom and the fulfillment of the diagnostic criteria was 4.67 ± 5.9 years. The duration between the time point of fulfillment of diagnostic criteria and the diagnosis (2.5 ± 2.1 years) was longer in patients having only mucocutaneous lesions (2.8 ± 2.2 years) than in patients having serious organ involvements (1.9 ± 1.6 years; p < 0.01). Serious involvements such as neurological involvement and large vessel involvement had their onsets later. Mean clinical severity score was higher in male patients (5.3 ± 2.1 vs 4.8 ± 1.7; p < 0.05). In logistic regression analysis, male gender (p = 0.03) and increased number of symptoms at diagnosis (p < 0.001, R (2) = 0.73) were found to be significant risk factors for severity. Mucocutaneous lesions, especially oral and/or genital ulcers, usually precede possible serious involvements; therefore, careful follow-up is mandatory. Males with increased number of organ involvements at the diagnosis are associated with more severe disease.

Rituximab is a beneficial and relatively safe adjuvant treatment for PV that facilitates prolonged clinical remission and has a significant steroid-sparing effect.

Background and Design: Pemphigus vulgaris (PV) is a severe, chronic, potentially life-threatening autoimmune blistering disease that affects the skin and mucous membranes, associated with the loss of cell-cell adhesion and blister formation. Systemic steroids in combination with immunosuppressive agents are the mainstay of therapy in pemphigus. Rituximab, a chimeric monoclonal anti-CD20 antibody, has been tried increasingly for the treatment of PV. Materials and Methods: We sought to test the efficacy and safety of rituximab as an adjuvant therapy by retrospective analysis of clinical and immunological data of patients. We performed a retrospective analysis of 13 patients with refractory pemphigus vulgaris who were treated with rituximab at Akdeniz University Hospital, Clinic of Dermatology and Venereology, Bullous Disease Unit. We evaluated clinical and immunological data with last treatments. Results: The patients were treated with one cycle of two biweekly infusions of rituximab at a dose of 1000 mg on days 1 and 15, except one received four doses of 375 mg/m 2 weekly. The mean follow-up time was 18.5 months. All patients had a decrease in antibody titers