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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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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