A significant number of patients who have chronic medical conditions often present with slow-healing wounds as a complication. Among the most serious medical conditions presenting with poorly healing wounds is calciphylaxis. This rare but life-threatening medical condition requires early diagnosis, and proper treatment by wound care professionals to improve patient outcomes.

What is Calciphylaxis?

Calciphylaxis is a serious disease that affects the blood vessels supplying the skin. Mechanical occlusion of the blood supply to the skin results in ischemia and skin tissue death manifesting physically as ulcerations on the skin’s surface.

How Does Calciphylaxis Develop?

Calciphylaxis is often described as a “heart attack” of the skin due to the similarities of its pathophysiology with cardiac arrest. The main causative factor in this condition is the deposition of calcium compounds within the small vessels supplying the skin. Unchecked calcification will lead to constriction and eventual occlusion of the blood vessel lumens. The resulting ischemia will deprive tissues in the affected area of essential nutrients, and oxygen leading to necrosis. As the disease progresses, calciphylaxis lesions worsen from obscure blood vessel injuries to visible epidermal, and dermal ulcerations.

Risk factors

There is a well-established link between patients with multiple chronic medical diseases and the development of calciphylaxis. Some risk factors for the development of vascular or epidermal lesions include:

• Chronic renal disease especially in patients with end-stage disease
• Sex with females at a higher risk than men
• Obesity
• Autoimmune diseases (e.g., lupus, rheumatoid arthritis, diabetes, and temporal arteritis)
• Abnormal elevations of blood calcium, and phosphate with calcium-phosphate complex deposition
• Hormonal dysregulation e.g hyperparathyroidism
• Hypercoagulable states precipitated by depleted vitamin C stores, as well as protein C and protein S deficiencies
• Metal toxicity (e.g., Iron and Aluminum)
• Medications (e.g., Calcium tablets, vitamin D, warfarin, insulin, corticosteroids, and immunosuppressive medications.

Although many of the aforementioned factors can result in the development of calciphylaxis, the single most critical determinant is the presence of end-stage renal disease. 

Clinical Presentation

Patients with calciphylaxis will typically present with the following complaints: 

• Rapid onset skin ulcerations (predominantly on the lower limbs)
• High fever
• Pain around ulcer sites

On examination, wound care professionals will note a “lacy” pattern to the ulceration, known as livedo reticularis which could be a collection of papules, plaques, or nodules with significant surrounding erythema (reddening). In more advanced lesions, a star-shaped pattern with a central area of tissue necrosis is easily recognizable. 

Diagnosis

The diagnosis of calciphylaxis obtained by combining the presence of suggestive clinical signs, and symptoms with laboratory investigations. Lab testing can be done using biopsy samples to assess for calcification within skin blood vessels or via blood samples to assay levels of various electrolytes and enzymatic components.  

Principles of Wound Care in Calciphylaxis

Treating patients with calciphylaxis requires a multi-disciplinary approach involving the efforts of an assortment of wound care specialists. General practice doctors, renal specialists, wound care nurses, dieticians, social workers, and physical and occupational therapists are all integral to wound management in calciphylaxis.

The principles of therapy include:

• Pain management
• Infection control
• Local wound care
• Nutritional support
• Psychological support
• Chronic disease management
• Other therapeutic options

Pain Management

The pain associated with calciphylaxis can be severe in some cases, with many patients needing analgesia to gain relief. Opioid analgesics, anticonvulsants, and non-steroidal anti-inflammatory drugs (NSAIDs) are indicated for use depending on severity, and side effect profile. 

Infection Control

Due to an increased risk of infection, patients with calciphylaxis will benefit from antibiotic therapy done in conjunction with local wound care. Wound swabs taken to isolate specific microorganisms should serve as a guide when prescribing antibiotics. 

Wound Care

Routine wound care practices like wound cleansing, debridement, and dressing are vital to the management of patients with ulcerations from calciphylaxis. In some cases, the debridement will be done surgically.

Nutritional Support

Nutritionists, and other wound care experts should counsel patients to restructure their diets. Foods with high calcium (milk, yogurt, cheese) and phosphorus (beer, cola, asparagus, nuts, spinach) should be limited and replaced with equally nutritious alternatives where possible.

Psychological support

Developing calciphylaxis can be a mentally traumatizing experience for patients. Psychological support therapy should be provided to all affected patients to help them cope with the emotional aspects of their condition, as well as help manage treatment expectations.

Chronic Disease Management

With chronic medical conditions such as renal failure, and diabetes mellitus among the key precipitants of the disease, wound care experts must ensure these conditions are adequately controlled using medical or surgical treatments. Well-controlled underlying conditions will improve the prognosis for patients with the condition. 

Other Therapeutic Options

Patients diagnosed with calciphylaxis may also benefit from newer therapeutic approaches that aim to aid wound healing and reduce blood calcium levels.

Examples include:

• Hyperbaric oxygen therapy
• Dialysis support with intravenous sodium thiosulphate
• Calcimimetics
• Bisphosphonates

Other Therapeutic Options

Patients diagnosed with calciphylaxis may also benefit from newer therapeutic approaches that could aid wound healing and reduce blood calcium levels.

Examples include:

• Hyperbaric oxygen therapy
• Dialysis support with intravenous sodium thiosulphate
• Calcimimetics
• Bisphosphonate