Treating Pain in a Patient with Calciphylaxis

Case Diagnosis: Calciphylaxis also known as calcific uremic arteriolopathy

Case Description or Program Description: 57yF with a PMH of type 2 diabetes mellitus, end-stage renal disease (ESRD) on hemodialysis, peripheral arterial disease, left below-knee-amputation (BKA), and heart failure presented initially with a right lower extremity wound eventually requiring an above-knee-amputation.

Setting: Acute Rehabilitation Hospital

Assessment/Results: On exam, the patient was noted to have multiple areas of skin hyperpigmentation on bilateral lower extremities that became progressively darker with a necrotic appearance and more painful. Skin biopsy (+) intraluminal calcifications suggestive of calciphylaxis. The patient's pain improved with a combination of topical steroids, NSAIDs in gel form, and oxycodone.

Discussion (relevance): Calciphylaxis occurs due to calcium phosphate depositions along the arterial wall leading to occlusion of micro-vessels thereby and subsequently painful skin lesions. These lesions suffer from poor healing evident through blistering, ulcerations, and necrotic tissue formation. Significant risk factors include recent skin injections, hyperphosphatemia, hyperparathyroidism, and Vitamin D deficiency commonly seen in dialysis patients.

Skin manifestations require prompt evaluation by a dermatologist or wound specialists and diagnosis can be confirmed by physical presentation or skin biopsy. Other differential diagnoses to consider include warfarin-induced skin necrosis, necrotizing vasculitis, or dystrophic calcinosis cutis. Treating pain in patients with calciphylaxis can be difficult due to certain limitations including the use of oral non-steroidal anti-inflammatory drugs in patients with renal dysfunction. Opioid analgesics can be utilized for severe pain; however, close monitoring is necessary due to risk of neurotoxicity. Non-opioid adjuvants should be strongly considered.

Conclusions: This case illustrates a severely debilitating disease process leading to impaired mobility secondary to skin manifestations and pain. An interdisciplinary approach is warranted with recommendations from nephrology, dermatology, wound care, palliative care, and pain management. Despite having an overall poor prognosis, acute inpatient rehabilitation can promote coordinated care in those with impaired mobility and skin healing due to calciphylaxis.