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Cutaneous porphyrias part II: treatment strategies

Identifieur interne : 001074 ( Main/Exploration ); précédent : 001073; suivant : 001075

Cutaneous porphyrias part II: treatment strategies

Auteurs : Suzanne Tintle [États-Unis] ; Ali Alikhan [États-Unis] ; Mary E. Horner [États-Unis] ; Jennifer L. Hand [États-Unis] ; Dawn Marie R. Davis [États-Unis]

Source :

RBID : ISTEX:A5D9A929E99FC3B0A392C790C8B83C43C2B7018F

Abstract

The porphyrias are diverse in pathophysiology, clinical presentation, severity, and prognosis, presenting a diagnostic and therapeutic challenge. Although not easily curable, the dermatological manifestations of these diseases, photosensitivity and associated cutaneous pathology, can be effectively prevented and managed. Sun avoidance is essential, and patient education regarding the irreversibility of photocutaneous damage is a necessary corollary. Beyond preventative measures, the care of fragile, vulnerable skin, and pain management, each of the porphyrias has a limited number of unique additional therapeutic options. Many of the treatments have been published only in small case series or anecdotal reports and do not have well‐understood nor proven mechanisms of action. This article presents a comprehensive review of available therapeutic options and long‐term management recommendations for the cutaneous porphyrias.

Url:
DOI: 10.1111/ijd.12016


Affiliations:


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Le document en format XML

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