What is immunobullous (autoimmune bullous) disease?

From the Guidelines

Immunobullous diseases, such as bullous pemphigoid, should be treated with immunosuppression using corticosteroids as first-line therapy, with the goal of reducing morbidity, mortality, and improving quality of life. The treatment of bullous pemphigoid typically involves the use of corticosteroids, such as prednisone, at a dose of 0.5-1.5 mg/kg/day, as recommended by the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology 1.

Key Considerations

• The disease is significantly associated with neurological disorders, and its morbidity and impact on quality of life are significant, making early diagnosis and treatment crucial 1.
• The use of steroid-sparing agents, such as azathioprine or mycophenolate mofetil, may be considered in certain cases to reduce the side effects of long-term corticosteroid use.
• Adjunctive treatments, such as dapsone or intravenous immunoglobulin, may be used in specific situations, but their use should be guided by the individual patient's needs and response to treatment.

Treatment Approach

• The treatment approach should be individualized, taking into account the patient's age, comorbidities, and the severity of the disease.
• Regular monitoring for medication side effects, including blood tests to check liver function, blood counts, and glucose levels, is essential to minimize the risks associated with immunosuppressive therapy.
• The goal of treatment is to achieve complete remission, or at least significant improvement, in the disease, while minimizing the risks of treatment-related complications.

Disease Pathophysiology

• Immunobullous diseases, including bullous pemphigoid, are characterized by the formation of autoantibodies against proteins essential for skin cell adhesion, leading to blistering of the skin and mucous membranes 1.
• The disease process involves the disruption of desmosomes or hemidesmosomes, which are protein complexes that hold skin cells together, resulting in the separation of skin layers and blister formation.

From the Research

Definition and Classification

• Immunobullous diseases are a group of blistering cutaneous disorders caused by pathogenic antibodies binding to protein targets within the skin 2.
• These diseases can be classified into two main categories: intraepidermal (pemphigus group of disorders) and subepidermal (pemphigoid group of disorders) 3.

Treatment Options

• The treatment of immunobullous diseases chiefly comprises corticosteroids and immunosuppressant adjuvants like azathioprine and mycophenolate mofetil 3.
• Adjuvant immunosuppressive drugs are widely used to minimize corticosteroid-related adverse effects in the short-term and long-term management of autoimmune bullous diseases 4.
• Rituximab, a chimeric anti-CD20 monoclonal antibody targeting B-cells, has emerged as an excellent treatment option for refractory pemphigus vulgaris 3.
• Other treatment options include cyclophosphamide, immunoadsorption, and high-dose intravenous immunoglobulin 5.

Efficacy and Safety of Treatment Options

• A 0·5 mg kg-1 per day dose of oral prednisone is a valuable therapeutic option in patients with mild or moderate bullous pemphigoid whose general condition allows them to be autonomous 6.
• Mycophenolate mofetil seems to be equally effective as azathioprine when used in combination with oral corticosteroids, but has a better safety profile 4.
• Cyclophosphamide still has a place in the treatment of severe relapsing autoimmune bullous diseases, but randomized controlled studies are needed to compare its efficacy and safety with newer treatment options 4.