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Initial management of pemphigus vulgaris and pemphigus foliaceus

Authors
Michael Hertl, MD
Shamir Geller, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

The term pemphigus describes a group of autoimmune mucocutaneous blistering disorders characterized by acantholysis (loss of keratinocyte to keratinocyte adhesion) in the epithelium of mucous membranes or skin. Pemphigus vulgaris and pemphigus foliaceus are the two most common forms of pemphigus. Significant morbidity and mortality can occur as a result of complications of these diseases and their treatments.

Systemic glucocorticoids are the mainstay of therapy for pemphigus vulgaris and pemphigus foliaceus, and are usually highly effective for obtaining control of disease. Nonsteroidal immunomodulatory agents such as azathioprine, mycophenolate mofetil, and dapsone commonly are prescribed in conjunction with systemic glucocorticoids in an attempt to minimize the risk for adverse effects of long-term, high-dose glucocorticoid therapy. Other interventions, including rituximab, intravenous immune globulin (IVIG), and cyclophosphamide are typically reserved for patients who fail to respond to these conventional therapies. (See "Management of refractory pemphigus vulgaris and pemphigus foliaceus".)

The initial management of pemphigus vulgaris and pemphigus foliaceus will be reviewed here. The management of refractory pemphigus vulgaris and pemphigus foliaceus, the pathogenesis and diagnosis of pemphigus, and the diagnosis and management of paraneoplastic pemphigus are reviewed separately. (See "Management of refractory pemphigus vulgaris and pemphigus foliaceus" and "Pathogenesis, clinical manifestations, and diagnosis of pemphigus" and "Paraneoplastic pemphigus".)

INDICATIONS FOR THERAPY

Although pemphigus vulgaris typically presents as a more severe disorder than pemphigus foliaceus due to the common presence of both mucosal and cutaneous involvement (picture 1A-B), both disorders can lead to significant morbidity. Oral mucosal involvement, which occurs in almost all patients with pemphigus vulgaris, usually is accompanied by severe pain, and may lead to poor alimentation resulting in weight loss and malnutrition (picture 2A-C). In addition, the widespread loss of the epidermal barrier that occurs in pemphigus vulgaris and pemphigus foliaceus may lead to protein loss, fluid loss, electrolyte imbalances, dietary insufficiencies, increased catabolism, and increased risk for local and systemic infections (picture 3A-D). (See "Pathogenesis, clinical manifestations, and diagnosis of pemphigus", section on 'Clinical features'.)

The complications of pemphigus vulgaris can be life-threatening; it is estimated that prior to the use of systemic immunosuppressants, more than 70 percent to nearly 100 percent of patients died within one to five years [1-3]. Pemphigus foliaceus, which is characterized by shallow blisters and involvement limited to the skin, is considered to have a better prognosis than pemphigus vulgaris (picture 4A-C) [3]; however, progression of pemphigus foliaceus may lead to extensive involvement and similar complications.

                           

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Literature review current through: Nov 2016. | This topic last updated: Tue Sep 22 00:00:00 GMT+00:00 2015.
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