The Porphyrias

Authored by: Eric Gou , Karl E. Anderson

Handbook Ofphotomedicine

Print publication date:  October  2013
Online publication date:  October  2013

Print ISBN: 9781439884690
eBook ISBN: 9781439884706
Adobe ISBN:

10.1201/b15582-15

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Abstract

Photosensitivity is a prominent feature of many of the porphyrias (Phillips and Anderson 2010). Chronic blistering lesions affecting sun-exposed skin occur in several types of porphyria and is the only clinical manifestation in porphyria cutanea tarda (PCT), the most common porphyria. In contrast, an acute, painful nonblistering photosensitivity occurs in erythropoietic protoporphyria (EPP), the third most common porphyria (Cox 2003; Sarkany 2008). These manifestations occur primarily from sunlight exposure. But especially in EPP, patients may be sensitive to fluorescent lighting and require accommodation at the workplace and at home. Also in EPP, operating-room lights during liver transplant surgery can cause marked phototoxic injury (Wahlin et al. 2008). Thus, knowledge of photomedicine is applicable to the understanding and management of these diseases.

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