P047 Successful treatment with dupilumab in patients with bullous pemphigoid and concomitant atopy

Abstract Dupilumab is a humanized antibody that inhibits interleukin (IL)-4/IL-13 signalling. It is approved for the treatment of multiple type 2 inflammatory diseases, including atopic dermatitis. The involvement of IgE antibodies and eosinophils also appears to be relevant in the pathogenesis of bullous pemphigoid (BP). The aim of this investigation was to scrutinize the efficacy and safety of dupilumab in patients with bullous pemphigoid and concomitant atopy. Six patients (four men and two women, average age 79.7 years) were hospitalized with eczematous lesions and intense pruritus. Diagnosis of BP was confirmed by direct and indirect immunofluorescence. All patients had a predisposition to atopy with an Erlangen atopy score ≥ 10 points. All patients were treated in label for atopic dermatitis with dupilumab at an initial dose of 2 × 300 mg then every 2 weeks, and were followed up for ≥ 3 months. No systemic pre-therapies had been initiated prior to treating BP. However, numerous comorbidities and comedications were known. The Bullous Pemphigoid Disease Activity Index showed an average value of almost 60 points. Complete remission of skin lesions was seen in three of six patients (50%) at week 4 and in five of six (83%) at week 12. A significant improvement in pruritus was observed in all patients (six of six), with half of the patients no longer experiencing itching after 4 weeks; this persisted for ≥ 12 weeks. All patients indicated a very high level of satisfaction, with an average of 5.0 (0 – very dissatisfied, 5 – very satisfied). No adverse events occurred during treatment with dupilumab. Together, the results suggest that IL-4/IL-13 signalling seems to be clinically important in the pathogenesis of BP, and may represent a promising target for BP therapy. As patients with BP are often older and present several comorbidities, dupilumab might be a promising treatment option with few side-effects.