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Therapeutic Approach

Pemphigus is a group of intraepidermal blistering diseases characterized by autoantibodies to the keratinocyte adhesion proteins desmoglein 3 (DSG3) and desmoglein 1 (DSG1), which cause flaccid blisters and erosions of the skin and/or mucous membranes. Major subtypes are pemphigus vulgaris (PV), which includes pemphigus vegetans and pemphigus foliaceus (PF), which includes pemphigus erythematosus. Therapy is based on the severity of disease and patient comorbidities. In the authors’ experience, topical therapy and/or doxycycline can be initiated for mild activity at disease onset, and systemic therapy with corticosteroids and rituximab are first-line therapy for moderate to severe disease, as well as any extent of disease that is relapsed or refractory to initial treatment. All theraipes for pemphigus in the table below are used off-label, with the exception of corticosteroids and rituximab.

Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB evidence includes evidence from ≥1 randomized controlled trial; level IIA evidence includes evidence from ≥1 controlled study without randomization; level IIB evidence includes evidence from ≥1 other type of experimental study; level III evidence includes evidence from nonexperimental descriptive studies, such as comparative studies, correlation studies, and case control studies; and level IV evidence includes evidence from expert committee reports or opinions or clinical experience of respected authorities, or both. See Table 38-1.

Table 38-1Pemphigus Treatment Table

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