Background Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering
disease of the skin and mucous membranes. This disease typically affects the elderly
and presents with itch and localized or, most frequently, generalized bullous lesions.
A subset of patients only develops excoriations, prurigo-like lesions, and eczematous
and/or urticarial erythematous lesions. The disease, which is significantly associated
with neurological disorders, has high morbidity and severely impacts the quality of
life. Objectives and methodology The Autoimmune blistering diseases Task Force of
the European Academy of Dermatology and Venereology sought to update the guidelines
for the management of BP based on new clinical information, and new evidence on diagnostic
tools and interventions. The recommendations are either evidence-based or rely on
expert opinion. The degree of consent among all task force members was included. Results
Treatment depends on the severity of BP and patients' comorbidities. High-potency
topical corticosteroids are recommended as the mainstay of treatment whenever possible.
Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of
contraindications or resistance to corticosteroids, immunosuppressive therapies, such
as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be
recommended. The use of doxycycline and dapsone is controversial. They may be recommended,
in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting
therapy and intravenous immunoglobulins may be considered in treatment-resistant cases.
Omalizumab and dupilumab have recently shown promising results. The final version
of the guideline was consented to by several patient organizations. Conclusions The
guidelines for the management of BP were updated. They summarize evidence- and expert-based
recommendations useful in clinical practice.
