32015 Practice patterns on the management of pemphigus – An international survey study

Abstract

Autoimmune blistering diseases (AIBD) such as pemphigus are characterized by circulating autoantibodies to desmosomal proteins which manifest as blisters and erosions of the skin and/or mucosa. Despite emerging diagnostic and therapeutic armamentarium, there remains considerable variability in the management of pemphigus. An anonymous survey was distributed to a group of bullous disease experts through the International Pemphigus and Pemphigoid Foundation. The χ2 and t tests were used for comparisons. P 75% of newly diagnosed and treatment naive pemphigus patients. Use of RTX first line was not associated with AIBD practice volume. Most (72.3%) prefer rheumatoid arthritis dosing regimen. 68.1% of respondents use desmoglein levels in assessing treatment response and need for retreatment. 63.8% of respondents provided concomitant systemic steroids, while 19.1% provided concomitant steroid-sparing agents to ≥90% of their patients on RTX. Few dermatologists (4.3%) routinely prescribe pneumocystis pneumonia (PCP) prophylaxis to all patients and only 34% prescribe PCP prophylaxis to some of their patients. Most (74.5%) do not routinely prescribe varicella zoster prophylaxis. 78.7% do not routinely administer prophylactic RTX for patients in clinical remission. Clinical relapse is the most common reason (76.1%) for RTX retreatment. This study highlights the variability in pemphigus management and the importance of further evidence-based guidelines for this condition.