TY - JOUR
T1 - Cutaneous porphyrias part II
T2 - Treatment strategies
AU - Tintle, Suzanne
AU - Alikhan, Ali
AU - Horner, Mary E.
AU - Hand, Jennifer L.
AU - Davis, Dawn Marie R.
PY - 2014/1/1
Y1 - 2014/1/1
N2 - 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.
AB - 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.
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U2 - 10.1111/ijd.12016
DO - 10.1111/ijd.12016
M3 - Review article
C2 - 24134210
AN - SCOPUS:84890790522
VL - 53
SP - 3
EP - 24
JO - International Journal of Dermatology
JF - International Journal of Dermatology
SN - 0011-9059
IS - 1
ER -