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Pemphigus

  • Chapter
Autoimmune Diseases of the Skin

Summary

Autoimmune bullous disorders of the pemphigus group present a major clinical challenge based on their severe clinical course and limited therapeutic options. Making the diagnosis of pemphigus requires histology, direct and indirect immunofluorescence and eventually, serological analyses with recombinant autoantigens. Currently, there is no consensus on generally accepted clinical partameters that help to measure disease activity and clinical responses or relapses. The therapeutic standard of pemphigus consists of a “global” non-specific immunosuppression with systemic glucocorticoids and immunosuppressive adjuvants causing severe side-effects and comorbidity. Due to the relative rareness of pemphigus, only few evidence-based controlled therapeutic trials have been performed. In light of limited approved immunosuppressive in the treatment of pemphigus, the “off-label-use” of potent immunosuppressive drugs is daily routine. Novel treatments, such as immunadsorption, rituximab and intravenous immunoglobulins bear the potential to induce clinical remissions in refractory pemphigus cases. The use of these drugs in pemphigus is described in recently published therapeutic guidelines

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  1. Department of Dermatology and Allergology, Philipp University Marburg, Deutschhausstraße 9, D-35037, Marburg, Germany

    Michael Hertl MD & Rüdiger Eming MD

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Hertl, M., Eming, R. (2011). Pemphigus. In: Hertl, M. (eds) Autoimmune Diseases of the Skin. Springer, Vienna. https://doi.org/10.1007/978-3-211-99225-8_3

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