Introduction: Calciphylaxis, also termed calcific uremic arteriolopathy (CUA), is a rare and severe vascular condition characterized by artery calcification and clotting, resulting in tissue ischemia and necrosis (1). It has traditionally been associated with end-stage renal disease, but there is growing evidence of its occurrence in individuals with underlying liver diseases, including nonalcoholic steatohepatitis (NASH) cirrhosis. The pathophysiological link between NASH cirrhosis and calciphylaxis remains elusive, but it is thought to involve a complex interplay of factors, including vascular calcification, hypercoagulability, and alterations in mineral metabolism (2). This case report highlights a unique instance suggesting a potential connection between NASH cirrhosis and calciphylaxis.

Case presentation: A 67-year-old male with PMH of NASH cirrhosis, diabetes mellitus, hypertension, hyperlipidemia, and obesity presented with a chief complaint of worsening painful lesions of bilateral lower extremities. Patient reports he initially noticed skin lesions about a month ago and has not yet been evaluated by Dermatology. Patient reported the lesions initially appeared as erythematous and violaceous nodules, which subsequently ulcerated. Over-the-counter pain medications did not adequately controlled his pain. On physical examination, the patient was noted to have multiple ulcerated, painful, and non-healing skin lesions on his lower extremities, starting from his bilateral shins. These lesions appeared to have dry central necrosis with eschar surrounded by a purpuric halo. The surrounding skin was indurated, and the lesions were tender to touch. No signs of infection were noted. Labs were remarkable for elevated LFTs, and decreased serum albumin indicating underlying cirrhosis. Serum electrolytes were unremarkable. Lower extremity Doppler ultrasound was negative for any deep vein thrombosis. Dermatology was consulted and a biopsy of the skin lesions was obtained.

Final working diagnosis: Calciphylaxis possibly secondary to NASH cirrhosis

Management/ outcome/follow-up: Given the patient’s painful and extensive skin lesions, the patient was started on opioid analgesics. Wound care team was consulted and local wound care including dressings along with topical antibiotics was initiated. Biopsy of the skin lesion resulted which showed Calciphylaxis.

During this hospitalization, the patient received multidisciplinary care with a focus on pain management, wound care, and daily labs to monitor electrolytes.

References and Resources

1. Gallo Marin, Benjamin, et al. “Calciphylaxis and kidney disease: A Review.” American Journal of Kidney Diseases, vol. 81, no. 2, 2023, pp. 232–239, https://doi.org/10.1053/j.ajkd.2022.06.011.
2. Markus, Johnathon MD; Ebrahim, Vivian MD; Wiland, Homer MD; Romero-Marrero, Carlos MD, FACG. Non-uremic Calciphylaxis in a Patient With NASH Cirrhosis: 431. American Journal of Gastroenterology 109():p S129, October 2014.
3. Gomes F, La Feria P, Costa C, Santos R. Non-Uremic Calciphylaxis: A Rare Diagnosis with Limited Therapeutic Strategies. Eur J Case Rep Intern Med. 2018 Dec 27;5(12):000986. doi: 10.12890/2018_000986. PMID: 30755998; PMCID: PMC6346971.