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Management of Bullous Pemphigoid

Recommendations for Immunomodulatory Treatments

  • Therapy in Practice
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Abstract

In 1953, Lever differentiated bullous pemphigoid from autoimmune pemphigus. The natural course of bullous pemphigoid is relatively benign, with a disease-related mortality rate of 24% compared with around 70% in pemphigus. In spite of the introduction of systemic corticosteroids, the mortality rates in bullous pemphigoid have generally not improved and vary between 0% and 40%. Higher doses of systemic corticosteroids seem to be associated with higher mortality rates, which led to the addition of corticosteroid-sparing agents to the treatment of bullous pemphigoid. However, many of these modalities are also accompanied by severe adverse effects and have not led to a significant decrease in the mortality rate. In recent years, there has been a move toward less toxic treatment options for a disease that is usually self-limited.

A systematic review of the literature found that treatment with lower doses of systemic corticosteroids and potent topical corticosteroids is effective and accompanied by less serious adverse effects, including death. No benefit of the addition of plasmapheresis or azathioprine to systemic corticosteroids has been shown. The treatment of bullous pemphigoid with tetracyclines and niacinamide (nicotinamide) is effective and accompanied by less serious adverse effects. However, more randomized controlled trials are needed to confirm these results and to determine the best treatment for bullous pemphigoid.

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Acknowledgments

The authors received no funding and have provided no conflicts of interest for the preparation of this manuscript. The treatment advice given in this manuscript is based on the results presented in the Cochrane review of the interventions for bullous pemphigoid1 and the British guidelines for the management of bullous pemphigoid.14 We thank Dr Marjolein Wintzen for critically reading the manuscript.

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Authors and Affiliations

  1. Department of Dermatology, Vrije Universiteit Medical Centre, PO Box 7057, 1007 MB, Amsterdam, The Netherlands

    Gudula Kirtschig

  2. Division of Dermatology, Groote Schuur Hospital and the University of Cape Town, Cape Town, South Africa

    Nonhlanhla P. Khumalo

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  1. Gudula Kirtschig
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  2. Nonhlanhla P. Khumalo
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Correspondence to Gudula Kirtschig.

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Kirtschig, G., Khumalo, N.P. Management of Bullous Pemphigoid. Am J Clin Dermatol 5, 319–326 (2004). https://doi.org/10.2165/00128071-200405050-00005

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