Cutaneous porphyrias part II: treatment strategies.

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.

journal_name

Int J Dermatol

authors

Tintle S,Alikhan A,Horner ME,Hand JL,Davis DM

doi

10.1111/ijd.12016

subject

Has Abstract

pub_date

2014-01-01 00:00:00

pages

3-24

issue

1

eissn

0011-9059

issn

1365-4632

journal_volume

53

pub_type

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