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Management of refractory pemphigus vulgaris and pemphigus foliaceus

Michael Hertl, MD
Rüdiger Eming, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD


Pemphigus vulgaris and pemphigus foliaceus are autoimmune blistering diseases that may result in significant morbidity and death. First-line treatment for pemphigus consists of systemic immunosuppression with systemic glucocorticoids with or without adjuvant immunosuppressive medications [1-3]. (See "Pathogenesis, clinical manifestations, and diagnosis of pemphigus" and "Initial management of pemphigus vulgaris and pemphigus foliaceus".)

Patients who do not respond to first-line therapies may benefit from additional interventions. Treatment regimens that include rituximab, intravenous immune globulin (IVIG), immunoadsorption, plasmapheresis, and cyclophosphamide may lead to clinical improvement in these patients.

The treatment of pemphigus vulgaris and pemphigus foliaceus refractory to initial therapies will be reviewed here. The initial management of pemphigus vulgaris and pemphigus foliaceus, and the management of paraneoplastic pemphigus are reviewed separately. (See "Initial management of pemphigus vulgaris and pemphigus foliaceus" and "Paraneoplastic pemphigus", section on 'Treatment'.)


The initial approach to the treatment of pemphigus vulgaris and pemphigus foliaceus consists of systemic glucocorticoid therapy, which is often given in conjunction with an adjuvant nonsteroidal immunosuppressant, such as azathioprine or mycophenolate mofetil. The term refractory pemphigus is generally used to refer to pemphigus that fails to respond sufficiently to optimal administration of these agents. The initial management of pemphigus is reviewed in detail separately. (See "Initial management of pemphigus vulgaris and pemphigus foliaceus".)

Various definitions for refractory pemphigus have been utilized in the medical literature. In 2008, a panel of experts proposed standardized definitions for the assessment of therapeutic responses in pemphigus for use in clinical studies, including definitions of treatment failure from certain therapies [4]. The consistent use of these guidelines in clinical studies may facilitate systematic interpretation of the literature in the future and may provide further clarification on therapeutic efficacy.

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Literature review current through: Nov 2017. | This topic last updated: Jul 20, 2017.
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