What is Calciphylaxis?
Calciphylaxis is a rare and serious disease characterized by an accumulation or deposition of calcium within the small vessels of the fat and skin tissues. It is a type of extraskeletal calcification and is usually secondary or specifically seen in patient suffering from stage 5 chronic kidney disease. The disease is generally characterized by necrosis of both the skin and fatty tissues and is manifested through painful skin ulcer that is non-healing while the condition is potentially life-threatening.
The etiology of calciphylaxis is mainly implicated in the calcium deposition within the small vessels of the fats and tissues. Calcium is an essential mineral that makes up the bones and teeth. It is also necessary for the function of the heart and nerves including the blood-clotting system of the body. The majority of the composition of both the teeth and the bones are made up of calcium. It is also essential for the bone breakdown and reconstruction that normally occur in the human body. Calcium is also necessary for muscle movements and is also an integral for nerves for its function in message transmission towards the brain. The required amount of calcium needed in the body depends on the age of an individual while sufficient amount is required for women and older people.
Calciphylaxis is an uncommon disease that is prevalent in women than in men. It can affect people of any race although it has been found prevalent in white people. Calciphylaxis can occur anytime from 6th month of life to 83 years of age with the mean onset at age 48 years. The disease is usually seen in individuals suffering from end stage kidney disease or to those undergoing dialysis. The prognosis is relatively poor with mortality linked to sepsis that resulted from necrotic skin lesions but may also be due to failure in internal organs.
Calciphylaxis is characterized based on the involvement of calcification and its characteristics such as:
- Systemic medial calcification of the arteries more commonly in the tunica media.
- Small vessel mural calcification either with or without the presence of endovascular fibrosis, vascular thrombosis and extravascular calcification that can result to tissue ischemia and skin ischemia or skin necrosis.
The onset of lesion is usually located in the fatty areas of the lower limbs. The lesion may also occur on the trunk, abdomen, thighs and buttocks although calciphylaxis on these areas tend to be more dangerous than the development of lesions in the lower limbs.
The general symptoms of calciphylaxis include the following:
- Retiform purpura or purplish colored mottling on the surface of the skin is the initial onset of calciphylaxis.
- Bleeding occurs on the site of retiform purpura.
- Blisters filled with blood may also develop.
- Unbearable pain and burning including itchiness may also be experienced over the affected site.
- Lesions are distributed either in the proximal or distal manner; proximal distribution is those located in the trunk, abdomen and thighs while distal distribution is lesions occurring below the knee.
- The center of the purplish lesion will eventually change to black color.
- Intense pain is unvarying in most cases of calciphylaxis.
The exact cause of calciphylaxis remains vague. The onset of the disease is primarily being linked to the end stage kidney disease and to patient undergoing dialysis. The link on renal failure is based on reported cases of calciphylaxis in patients suffering from long-term renal failure and renal replacement therapy. It is very rare for calciphylaxis to occur in a patient without any history of kidney disease or end stage renal failure.
Diseases and disorders of the kidney are mostly implicated in the onset of calciphylaxis while other factors are also being considered to the incidence.
Chronic renal failure
Chronic renal failure is the progressive loss of kidney function overtime. Stage 5 of the disease is the most severe case with poor prognosis of mortality. The stage is also known as the end stage renal failure where during this stage calciphylaxis is known to occur. Chronic renal failure is the most implicated in the incidence of calciphylaxis.
Hypercalcemia is an elevation in the levels of calcium in the blood as a consequence of overactivity in one or more of the parathyroid glands that is responsible for regulating the level of calcium in the blood. This is another condition that is also being linked to the incidence of calciphylaxis.
Hyperparathyroidism is an excessive parathyroid hormone in the blood as a consequence of hyperactivity in the parathyroid glands. Excessive levels of parathyroid hormone are potentially harmful to the bones while this condition is being linked to the onset of calciphylaxis.
Crohn’s disease is among the disorders included in the case reports of calciphylaxis. Crohn’s disease is an inflammatory bowel disease characterized by an abdominal pain and severe diarrhea including malnutrition.
Diabetes mellitus is also another chronic disorder that is being linked or considered a risk factor for calciphylaxis as a result of direct trauma to the subcutis as brought by insulin injection.
Other risk factors considered potentials for calciphylaxis include the following:
- Obesity or overweight.
- An individual suffering from diabetes.
- Exposure to immunosuppressive agents such as glucocorticoids and other agents such as glucocorticoids, albumin infusion and iron dextran complex.
Medical history taking is the initial step in diagnosing calciphylaxis through obtaining information relevant to the onset of the condition. The physical exam is the next step in diagnosing calciphylaxis through clinical presentation of the lesions.
Blood tests may be considered in the diagnostic procedure in measuring numerous substances found in the blood such as calcium and parathyroid hormone.
This may be done to clearly define the onset of calciphylaxis and isolate it from other conditions that might have similar symptoms with calciphylaxis.
Imaging tests such as CT scans, bone scans, mammography and x-rays can help in diagnosing calciphylaxis through revelation of vascular calcifications which is indicative of calciphylaxis.
Treatment for calciphylaxis remains obscure while there is no treatment or cure directed towards the condition. The aim of treatment focuses on the existing underlying condition that influenced the incidence of calciphylaxis. The aggravating condition must be addressed promptly while elimination is advisable for triggering factors. Both medical and surgical intervention can be utilized in treating and managing calciphylaxis.