What is Calciphylaxis ?

 CALCIPHYAXIS 

Calciphyaxis is a rare , painful and deadly disease that is most likely to be seen in people with moderate to severe kidney problems . When causes calcium deposits form in your blodd vessels and block blood flow  to areas of  your skin . This can lead to open wounds that are prone to dangerous infections . 
Calciphyaxis  disease causes calcium deposits to form in your blood vessels and block blood flow, leading to areas where skin and tissues just underneath break down and die . Calciphyaxis  is a rare but serious disease, believed to affect 1-4 % of all dialysis patients . Calciphyaxis is one type of extraskeletal calcification . Extreskeletal calcifications are obsered in some people with  high   levels of calcium in the blood , including people with milk - alkali  syndrome , sarcoidosis , primary hyperparathyroidism , and hypervitaminosis D . 

CALCIPHYAXIS  PICTURES :




HOW TO TREAT CALCIPHYLAXIS ?

The treatments of calciphyaxis requires a multidisciplinary approach  , using the knowledge of nephrologists , plastic surgeons , dermatologists , and wound care specialists working together  to manage the disease  and its outcomes. The key to treating  calciphylaxix  is prevention via rigorous control of phosphate and calcium  balance and management  of risk factors in patients  who have increased chances of developing calciphylaxis . It generally accepted to apply a multi pronged approach to each patients . 


ANALGESIA AND WOUND MANAGEMENT :

Pain  management and choice of analgesia is a challenging task in managing calciphylaxis . pain is one of the most severe and pervasive symptoms of the disease and can be unresponsive to high - dose opioids . Care for calciphylaxis lesions  involves using approprites dressings , wound debridement , and prevention of infection . Infection leads to sepsis , one of the leading causes of death in patients with calciphylaxis . There are some smaller retrospective studies that show the use of hyperbaric oxygen in improving delivery of oxygen to wounds , which improves blood flow and helps with wound healing . 

RISK FACTOR MITIGATION :

Most patients with calciphylaxis are already on hemodialysis , or simply dialysis , but the length or frequency of sessions may be increased . The  majority  of dialysis patients are on 4 - hour three times per weeks  schedule . A disease statte that causes low calcium and requires use of calcium supplementations and calcitriol, which should be avoid in patients with end stage  kidney  disease and calciphylaxis . 

CAUSES OF CALCIPHYLAXIS :

The cause of calciphylaxis i sunknown . Calciphylaxis is not a hypersensitivity  reaction  leading to sudden local calcification . The disease is also  known as calcific uremic arteriolopathy   the disease is not  limited to patients with kidney failure . The calcium has been deposited  , event occur within the  lumen of these  vessels , resulting in occlusion of the vessels and subsequent tissues infarction . Another theory has been  proposed  that vitamin K deficiency due to dietary restrictions meant to limit potassium and sodium . Many end stage kidney disease  patients are also on a medication  called warfarin , a vitamin K antagonist , that limits vitamin k recycling in the body . Patients who require or have  undergone any type of vasular procedures are also at increased risk for poor outcomes .  

DIAGNOSIS FOR CALCIPHYLAXIS :

There is no diagnostic test for calciphylaxis . the diagnosis is a clinical one . The characteristics lesions are the ischemic skin lesion . The necrotic skin lesions typically appear  as violaceous lesions and completely black leathery lesions . The suspected diagnosis can be supported by a skin biopsy , usually a punch of biopsy , which shows  arterial calcification and occlusion in the absence of vasculitis . In certain patient  an anti nuclear antibody test may play a role in diagonsis of calciphylaxis . Plain radiography  and mammography may also show calcification but these  tests are less sensitive .  Laboratory studies , such as phosphate levels , calcium  levels, and parathyroid levels , are nonspecific and unhelpful for diagnosis of calciphyaxis . 



PROGNOSIS FOR  CALCIPHYLAXIS :

The clinical prognosis for calciphylaxis  is poor . The 1 - year mortality rate in patients who have end stage kidney disease is 45- 80 % . Median survival in patients who do not have ends - stage kidney  disease is 4.2 months . Reponse to treatment is not guaranteed . The most common cause of death in calciphylaxis patients  is sepsis , severe infection originating from a non - healing ulcer. 

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