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Blistering Disorders (Porphyria Cutanea Tarda and Pseudoporphyria)

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Dermatological Manifestations of Kidney Disease

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Abstract

Porphyria cutanea tarda (PCT) and pseudoporphyria represent the most common subtypes of porphyria presenting in patients with end-stage renal disease (ESRD). In these patients, there is a disorder of heme synthesis that produces a pathogenic accumulation of photosensitizing intermediary metabolites. The result of this disorder is a photodistributed pruritic blistering eruption as well as other characteristic clinical and laboratory findings. The distinction between PCT, pseudoporphyria, and other vesiculobullous disorders may be challenging. Thus, the aim of this chapter is to provide a comprehensive overview of the features, diagnostic markers, and treatment regimens for these common cutaneous entities observed in individuals with concurrent renal dysfunction.

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Abbreviations

ESRD:

End-stage renal disease

HBV:

Hepatitis B virus

HCV:

Hepatitis C virus

HIV:

Human immunodeficiency virus

PAS:

Periodic acid-Schiff

PCT:

Porphyria cutanea tarda

UROD:

Uroporphyrinogen III decarboxylase

UVA:

Ultraviolet A

UVB:

Ultraviolet B

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Authors and Affiliations

  1. Department of Dermatology, Georgetown University/Washington Hospital Center, 5530 Wisconsin Ave, Suite 660, Chevy Chase, MD, 20815, USA

    Christine A. DeWitt M.D.

  2. Georgetown University School of Medicine, 3900 Reservoir Road NW, Washington, DC, 20057, USA

    Kara M. Trapp B.A.

  3. Department of Dermatology, Georgetown University Hospital/Washington Hospital Center, 110 Irving St. NW, 2B-44, Washington, DC, 20010, USA

    Melissa A. Muszynski M.D.

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  1. Christine A. DeWitt M.D.
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  2. Kara M. Trapp B.A.
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  3. Melissa A. Muszynski M.D.
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Correspondence to Christine A. DeWitt M.D. .

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Editors and Affiliations

  1. Department of Dermatology, Nelson Clinic, Virginia Commonwealth University Medical Center, Richmond, Virginia, USA

    Julia R. Nunley

  2. Department of Medicine, Section of Nephrology, University of Illinois at Chicago / Advocate Christ Medical Center, Oak Lawn, Illinois, USA

    Edgar V. Lerma

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DeWitt, C.A., Trapp, K.M., Muszynski, M.A. (2015). Blistering Disorders (Porphyria Cutanea Tarda and Pseudoporphyria). In: Nunley, J., Lerma, E. (eds) Dermatological Manifestations of Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2395-3_13

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