Rituximab and low dose oral immune modulating treatment to maintain a sustained response in severe pemphigus patients.

Objectives: This pilot retrospective study examined the role of continuous low-dose maintenance immunomodulatory treatment (IMT) as an adjunct to rituximab (RTX) rescue therapy in severe pemphigus vulgaris (PV) and pemphigus foliaceus (PF) after a complete response (CR) to RTX was achieved. Materials and methods: Ten severe pemphigus patients who received RTX rescue therapy were evaluated after achieving CR. The post-RTX clinical course and long-term follow up was compared between patients who adhered to a low recommended dose (LRMD) to patients who were non-compliant with LRMD. Results: Five patients relapsed due to discontinuing or tapering their LRMD therapy, whereas the five patients who adhered to their maintenance therapy did not experience a relapse after the initial post-RTX CR. A combination of increasing or adding IMTs and initiating subsequent RTX cycles was used to regain control in relapsed patients. Conclusions: We propose an alternative treatment strategy utilizing RTX as a rescue agent in combination with long-term LRMD as a means to maintain a sustained long-term CR post-RTX therapy in severe pemphigus patients. This strategy could prevent disease flares and the need for additional RTX cycles and higher dosages of immunomodulatory therapies.

Perforating disorder secondary to leflunomide and review of the literature of medications associated with perforating disorder.

The perforating dermatoses are a group of disorders characterized by transepidermal elimination of a material from the upper dermis. The most widely accepted classification consists of four primary perforating disorders that are defined by the type of material eliminated and the type of epidermal disruption. Pathogenesis of the perforating dermatoses is poorly understood, but some appear to have a genetic component. There are also acquired forms, which have been associated with underlying systemic diseases and the use of certain drugs. In this report, we describe a perforating disorder that occurred secondary to leflunomide therapy. To our knowledge, this is the first case in which this has been reported. We also review the recent literature on medications associated with perforating disorders.

Oral lichen sclerosus-a review of clinical presentation, treatment, and clinical outcomes.

OBJECTIVE: The aim of this study was to evaluate the disease involvement, treatment and response, and malignant potential of oral lichen sclerosus (LS). STUDY DESIGN: We conducted a review of the literature of 37 cases of oral LS. We looked specifically for the following data: patient demographic characteristics, disease involvement, treatment, response to treatment, duration of follow-up, symptoms, risk factors for oral malignancy, and malignant transformation. RESULTS: The most common area of oral involvement included the labial mucosa (67.6%). Of patients with oral LS, 38.5% reported symptoms, and 35.1% exhibited extraoral manifestations. Patients were less likely to receive treatment when asymptomatic (62.5%) than when symptomatic (80%). Topical steroids were more efficacious (66.7%) compared with other treatments. Risk factors for malignancy were found in 25% of patients. No patient had malignant transformation of oral lesions at follow-up (mean follow-up 22.1 months; median 12 months). CONCLUSIONS: More data and longer follow-up are required to determine the long-term clinical outcomes of oral LS.