Current Treatment of Autoimmune Blistering Diseases

Abstract

Autoimmune bullous diseases are a heterogeneous group of disorders that can be subdivided according to the level of split formation in the intraepidermal blistering pemphigus diseases and subepidermal bullous disorders, latter including pemphigoid diseases, epidermolysis bullosa acquisita, and dermatitis herpetiformis. In the majority of autoimmune bullous disorders, disease activity can be sufficiently controlled by systemic corticosteroids in combination with further immunosuppressants/immunomodulants such as dapsone, doxycycline, methotrexate, azathioprine, or mycophenolate mofetil. In contrast, in pemphigus, mucous membrane pemphigoid, and epidermolysis bullosa acquisita, treatment is challenging and only in a minority of patients, conventional immunosuppressive therapy induces clinical remission. Since only a few years ago, only cyclophosphamide and high-dose intravenous immunoglobulin were available as potent second-line therapies. Meanwhile, immunoadsorption and the monoclonal anti-CD20 antibody rituximab have been established as further therapeutic options. Here, both conventional therapies and novel treatment regimens for autoimmune blistering diseases are discussed.

Role of Corticosteroids in Oral Lesions

“…5mg/kg/day) is added. Instead of dapsone, doxycycline (200mg/day) may be given (Kasperkiewicz 2009). …”

Peptide-mediated desmoglein 3 crosslinking prevents pemphigus vulgaris autoantibody-induced skin blistering

“…Despite the variety of possible therapeutic approaches under experimental conditions, the therapy of pemphigus patients still is based on long-term use of high-dose systemic corticosteroids in combination with other immunosuppressants including azathioprine, mycophenole, cyclophosphamide, and more recently, rituximab (27,28). Due to the frequently observed adverse reactions, novel and specific treatment options are highly needed.…”

Immunotherapies in Dermatologic Disorders

“…Some patients achieved partial remission and enhanced/improved quality of life and were able to decrease their corticosteroid dosages when using rituximab. [145][146][147] Dermatomyositis is an idiopathic inflammatory disease with B-cell perivascular infiltrates; rituximab has been used for treatment of refractory cases. Patients with dermatomyositis demonstrated improvement when treated with rituximab.…”