Updates on the Management of Autoimmune Blistering Diseases

Joanna N. Hooten, MD; Russell P. Hall 3rd, MD; Adela R. Cardones, MD

Disclosures

Skin Therapy Letter. 2014;19(5) 

In This Article

Abstract and Introduction

Abstract

Autoimmune blistering diseases are rare, but potentially debilitating cutaneous disorders characterized by varying degrees of mucosal and cutaneous bullae formation. Topical therapy is appropriate for mild and even some moderate disease activity, but systemic treatment can be considered for more extensive involvement. Corticosteroids remain the first-line systemic therapy for patients with moderate to severe bullous pemphigoid and pemphigus vulgaris. While the use of systemic steroids has dramatically reduced mortality from these two autoimmune blistering disorders, treatment is also associated with multiple side effects, especially when used long-term. Steroid sparing agents, therefore, are invaluable in inducing long-term remission while minimizing steroid associated side effects. Treatment must be tailored to the individual patient's condition, and several other factors must be carefully considered in choosing appropriate therapy: 1) diagnosis, 2) severity of the condition and body site affected, 3) presence of comorbidities, and 4) ability to tolerate systemic therapy.

Introduction

Autoimmune blistering diseases (AIBD) are a heterogeneous group of chronic, acquired disorders characterized by blister formation within the epidermis, at the dermal-epidermal junction or at the basement membrane zone, and by the presence of autoantibodies directed against structural components of cellular adhesions molecules. AIBD are classified into different groups based on clinical and immunopathological criteria. Largescale, randomized studies on effective therapeutic strategies for AIBD are limited and treatment varies widely among providers. The patient population tends to be a heterogeneous mix of age, gender and comorbidities, and the mechanisms of injury to the skin are variable between blistering diseases. Management of these disorders, therefore, requires an understanding of the spectrum of available therapies. A complete discussion of the treatment of AIBD is beyond the scope of this review, however, we present some recommendations in approaching patients with pemphigus, pemphigoid, and epidermolysis bullosa acquisita (EBA). A brief description of the available medications and their roles in treating AIBD will be provided, but a thorough review of therapeutic and monitoring guidelines must be done before treatment is initiated.[1]

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