Search

Introduction : This review assesses current evidence supporting dose de-escalated rituximab therapy in pemphigus vulgaris, compared to standard protocols. Primary outcome measures were remission and relapse rates. Adverse effects, cumulative steroid dosages, and serological markers of disease activity were also reported. Areas covered : A literature search was performed to look for reports describing the use of de-escalated rituximab therapy in pemphigus vulgaris. Results from heterogenous studies showed a large variation in remission and relapse rates. Complete remission rates from de-escalated treatment ranged from 41.7 to 100.0%, while rates in the control groups ranged from 60.0 to 90.9%. Relapse rates varied from 8.0 to 81.3% in the de-escalated group and from 0.0 to 72.4% in the control group. Of the 165 patients included in this report, only two major adverse effects were reported. Expert Opinion : Overall, dose de-escalated rituximab protocols reported to date appear effective and safe. However, it is unclear if treatment effect parallels that of standard regimens in regard to disease control in the long term. A lower limit of effective dosing for rituximab in pemphigus vulgaris has not yet been reached or defined. The role for and timing of repeated cycles of low-dose rituximab therapy require further exploration.

Pemphigus vulgaris (PV) is an auto-immune blistering skin disease characterized by flaccid blisters and painful erosions of mucous membranes and skin. Suprabasal blister formation results from a loss of epidermal cohesion induced by auto-antibodies against the desmosomal protein desmoglein 3. Treatment of PV currently consists of systemic glucocorticosteroids and adjuvant immunosuppressive drugs such as azathioprine, mycophenolate mofetil, cyclophosphamide or dapsone. Due to the low incidence of PV, there are insufficient data to conclude which treatment is the most effective and safest. Thus far, systemic tacrolimus (FK506, Prograf) has not yet been reported as adjuvant medication for PV. Here, we describe the successful use of systemic tacrolimus in 2 patients with recalcitrant PV of the oral mucosa. Tacrolimus was well tolerated, and clinical improvement allowed tapering of corticosteroids. Thus, oral tacrolimus may be a therapeutic alternative for patients with recalcitrant PV., (Copyright 2010 S. Karger AG, Basel.)

Background: The BIOCHIP is an indirect immunofluorescence diagnostic investigation which identifies multiple autoantibodies with a mosaic panel of target antigen‐specific substrates in a single incubation field. The EUROIMMUN Dermatology Profile ELISA allows simultaneous investigation of the six most important autoantibodies in bullous autoimmune dermatoses. Evaluation of the BIOCHIP Mosaic 7, compared to that of the EUROIMMUN Dermatology Profile ELISA, when used as a diagnostic investigation in pemphigus and pemphigoid, was undertaken in an Australian cohort. Methods: The serum of 27 patients was analysed including patients with pemphigus vulgaris (n = 10), pemphigus foliaceous (n = 4), bullous pemphigoid (n = 8), mucous membrane pemphigoid (n = 3) and negative controls (n = 2). Results of the BIOCHIP were compared with the EUROIMMUN Dermatology Profile ELISA, as well as with histology, direct immunofluorescence and indirect immunofluorescence. Results: In pemphigus vulgaris, sensitivity & specificity for the BIOCHIP Mosaic 7 were 100% and 94.1%, comparable to that of the EUROIMMUN Dermatology Profile ELISA with 80% sensitivity and 100% specificity. In bullous pemphigoid, sensitivity of the BIOCHIP was 87.5% and sensitivity of the EUROIMMUN Dermatology ELISA profile was 75%, whilst specificities for both diagnostic methods were 100% in our limited cohort. There was substantial or almost perfect concordance between the BIOCHIP Mosaic 7 and EUROIMMUN Dermatology Profile ELISA for pemphigus vulgaris and bullous pemphigoid. Conclusion: The BIOCHIP Mosaic 7 is a rapid, reliable diagnostic investigation in pemphigus and bullous pemphigoid. Results indicate it is comparable to the EUROIMMUN Dermatology Profile ELISA, whilst also providing additional testing with salt split skin, on one field. [ABSTRACT FROM AUTHOR]