XXXX : a still unmet challenge.

@entity1 @entity1066 @entity740 @entity730 @entity281 @entity272 @entity13375 @entity65
INTRODUCTION: @entity730 ( @entity730 ), also known as @entity13375 , is a rare disease most frequently occurring in @entity1 with @entity740 ( @entity740 ). The clinical picture is typically characterized by very painful @entity272 and @entity1066 following calcification and occlusion of small cutaneous arterioles. @entity730 is life-threatening due to @entity281 and concomitant @entity65 . METHODS: We performed a literature search for the terms @entity13375 and @entity730 and summarized current state-of-the-art knowledge about pathophysiology, clinical picture, course of the disease, as well as treatment options. We have filled out the literature data with our personal treatment experiences. RESULTS: A combination of various local and systemic risk factors are necessary to cause the development of @entity13375 . This pathophysiological cascade is still incompletely understood. @entity1 with advanced @entity740 and dialysis @entity1 are especially at risk to develop @entity730 . Regarding therapy, no randomized prospective trials are available, and treatment is rather based on pathophysiological considerations as well as on evidence derived from case reports or case series. Therapy focuses on optimized dialysis treatment, control of @entity740 parameters, experimental anticalcification strategies and wound care. CONCLUSION: Facing the still deleterious outcome of @entity1 with @entity13375 , further studies on prophylaxis as well as treatment are urgently needed. Current treatment strategies may help ameliorate the course of the disease in some @entity1 . However, it is still unclear if they are able to decrease mortality.
