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Calciphylaxis: no therapeutic concepts for a poorly understood syndrome?
Author(s) -
Meissner Markus,
Gille Jens,
Kaufmann Roland
Publication year - 2006
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/j.1610-0387.2006.06127.x
Subject(s) - calciphylaxis , medicine , intensive care medicine , therapeutic approach , disease , sepsis , sodium thiosulfate , pathogenesis , ischemia , amputation , bioinformatics , dermatology , surgery , pathology , chemistry , inorganic chemistry , biology
Summary Calciphylaxis is a very uncommon and severe disease which mainly appears in patients with chronic renal insufficiency. It presents with ischemia and necrosis of the skin, subcutaneous adipose tissue, muscles and rarely viscera.The pathogenetic mechanisms inducing calciphylaxis are for the most part unknown.The mortality rate of 80% in the first year is very high. Patients experience marked pain, recurrent infections and the constant risk of secondary sepsis. Even multidisciplinary therapeutic strategies are limited, although there are recent case reports providing promising new therapeutic options including sodium thiosulfate and cinacalcet. This review summarizes the important aspects of diagnosis, pathogenesis, prevention and the possible therapeutic strategies of this intriguing, rare and often fatal disease.