It is a rare but very serious disease which causes calcium to accumulate in the small blood vessels of skin tissues and fat. It is characterized by thrombosis, vascular calcification and skin necrosis. The condition is almost exclusive in people suffering from Stage 5 Chronic Renal Disease or End Stage Renal Disease. However, it may also occur in people without the kidney disease.
It is usually fatal, causing non-healing wounds or lesions.
The disorder is also referred to by various other names like:
- Ischemic Necrosis Skin-Muscles
- Calcific Uremic Arteriolopathy
- Idiopathic Calciphylaxis
This condition mainly occurs in dialysis patients with serious renal disorders. The exact prevalence statistics is not known. However, experts believe that 1 out of every 1000 to 1500 patients with chronic renal disorders develop Ischemic Necrosis Skin-Muscles in a year.
The exact etiology of this disease is still unknown. According to experts, the lesions are not the result of some immune type reaction. It is not an allergic or hypersensitivity reaction, causing sudden local calcification. Individuals with chronic renal failure are at great risk of developing the condition.
Some case studies suggest that Ischemic Necrosis Skin-Muscles might have an association with Rheumatoid Arthritis. Some additional factors are believed to be involved with the occurrence of the wounds. These include:
- Weight loss
- Kidney transplantation
- Calcification in various blood vessels
- Use of corticosteroids
- Use of anticoagulant like warfarin (Coumadin)
Calciphylaxis Signs and Symptoms
Patients of this condition suffer from various health problems and skin disorders due to the development of painful ulcers. The condition is characterized by:
- Multiple organ failures
- Certain skin infections
- Continuous burning and itching sensations
- Bleeding from the area primarily involved
- Several red or purple lesions on the abdomen, trunk, buttock and thigh
- Systemic medial calcification of arteries (such as the tunica media)
- Calcification of the small vessel walls (endovascular fibrosis may or may not be present)
- Possible development of fluid-filled lesions
- Tissue ischemia, which leads to cell necrosis
Doctors generally perform a detailed physical exam and thoroughly study the medical history of a patient for diagnosing the lesions. Various diagnostic tests may be used, which include the following:
Deep Skin Biopsy
Sometimes, doctors may order a skin biopsy in which a tissue sample is collected from the affected skin area and sent for analysis.
It is done to identify and measure a number of substances, including phosphorus, calcium, aluminum, parathyroid hormone, urea nitrogen, albumin and creatinine, in the blood of patients. This helps a diagnostician to assess the functioning of the liver and kidney.
Various imaging tests are useful for making this diagnosis. X-ray is performed for observing the vascular calcifications that commonly occur in this condition. However, this symptom can also be present if a patient is suffering from some form of advanced kidney disease. Due to this reason, additional imaging studies are also required for confirming the diagnosis. These involve:
- Bone scintigraphy (bone scans)
- High-resolution CT scans
Calciphylaxis Differential Diagnosis
While detecting this disease, a diagnostician should rule out the possibility of the following skin conditions:
- Bullous Pemphigoid
- Vibrio Vulnificus Infection
- Wegener Granulomatosis
- Erythema Nodosum
- Hypersensitivity Vasculitis or Leukocytoclastic Vasculitis
- Bullous Lupus Erythematosus
- Pyoderma Gangrenosum
- Venous ulcers
- Necrotizing Fasciitis
Calciphylaxis Treatment and Management
There is no standard treatment procedure for managing these lesions. Treatment varies depending on the stage of the condition and existence of any associated disorders. It is important to begin the treatment at an early stage to achieve a positive outcome.
Intensive Wound Care
The initial stage of the treatment is intensive care of the wounds. In some cases, the tissues damaged by the condition require surgical removal (debridement). Sometimes, other procedures including wet dressings and whirlpool treatments are also used for removing the damaged tissues. Antibiotic treatment is necessary to prevent and treat wound infections. A doctor may prescribe certain medications for pain management. Other treatment measures include:
Restoring Blood and Oxygen Flow
Hyperbaric oxygen therapy may be recommended by a doctor for increasing the oxygen flow to the affected regions. Sometimes, a low-dose TPA (tissue plasminogen activator) may also be used for dissolving the blood clots occurring in the small blood vessels of skin. Blood thinning medications can help prevent the formation of additional blood clots.
Decreasing Calcium Deposits
The following procedures can help to reduce the calcium deposits in the arteries:
A doctor may change the dialysis prescription if an affected individual is on kidney dialysis. The frequency of dialysis and the medications used by the patient are generally changed for this treatment.
Drugs that help to reduce the calcium deposits are often prescribed by a physician. He or she also evaluates all the medicines used by a patient to check if any of those medicines are responsible for triggering the lesions. Corticosteroids and iron medications are among these precipitating factors. Doctors may also modify the dosage of any vitamin D and calcium supplements. Cinacalcet (Sensipar) is sometimes prescribed for controlling the parathyroid hormone (PTH) level of a sufferer. Sodium thiosulfate is another medicine which is commonly used in this treatment. Medications may also be prescribed for improving the calcium and phosphorus balance in the body.
Sometimes, a hyperactive parathyroid gland (hyperparathyroidism) produces excessive amounts of PTH thus leading to abnormalities in calcium metabolism. In such cases, surgery is recommended for partial or complete removal of the patient’s parathyroid glands.
Individuals suffering from Ischemic Necrosis Skin-Muscles generally have a poor prognosis. The location of the wounds is a deciding factor in the outcome of the treatment. In most cases, the wound leads to serious infection and organ failure. This often leads to the death of patients. Timely treatment may make it possible to save a patient’s life. However, complete cure might require limb amputation and various reconstructive surgeries which can consequently result in considerable physical disability.
The disease can lead to several serious complications, unless treated properly. The common complications include ulceration and pain in different areas on the skin as well as blood infection. A patient may also undergo severe lung calcification. The lesions may turn fatal due to multi-organ failure and infection.
Calciphylaxis Life Expectancy
Patients of this disease usually have a very short lifespan. In severe cases, the life expectancy of sufferers is no more than six months. The condition is associated with a mortality rate ranging between 60% and 80%. However, use of modern treatment procedures has been found to contribute to a reasonably high survival rate.
This is an incurable and fatal condition, which means preventing the occurrence of the disorder is very important for individuals at risk of developing these lesions. Controlling the calcium and phosphate balance strictly and regularly can help to prevent the metabolic changes that may cause Calciphylaxis. Avoiding serious skin trauma and taking early care of local wounds is also important for preventing this disorder.
Calciphylaxis Support Groups
A number of forums and foundations are spreading awareness and providing guidelines regarding this disorder so that patients are able to take care of the lesions. These include:
The Renal Support Network
1311 N. Maryland Avenue
Glendale, California 91207
Phone: (818) 543-0896
Fax: (818) 244-9540
The Renal Association
Hampshire, GU31 5AZ
Phone: 0845 1800 484
Fax: 01730 715291
Check out some images that provide useful visual information about the lesions arising due to Ischemic Necrosis Skin-Muscles.
Picture 1 – Calciphylaxis
Picture 2 – Calciphylaxis Image
As aforesaid, Calciphylaxis is a rare but life threatening condition which can lead to cell necrosis in different body parts along with multiple organ damage. Naturally, early diagnosis and prompt treatment is essential to increase chances of survival for patients of this disorder.