A0554 - Recognizing Deadly Skin Lesions in a Post Liver Transplant Patient: A Survival Story

Sunday, October 23, 2022

5:00 PM – 7:00 PM ET

Location: Crown Ballroom

Presenting Author(s)

Aldanah Althwanay, MD

University of Maryland Medical Center
Baltimore, MD

Aldanah Althwanay, MD, Sassan Sakiani, MD
University of Maryland Medical Center, Baltimore, MD

Introduction: Calciphylaxis is a syndrome where calcium accumulates in vessels of the fat and skin, leading to necrosis, infections, and potentially death. The one year mortality is more than 50%. There are two types: uremic and non-uremic (NUC). The latter is rare and has poorly understood associations, such as Caucasian race, female sex, connective tissue disease, and medications such as warfarin and steroids. In the literature thus far, there are only 3 reported cases of NUC post orthotopic liver transplantation (OLT) with varied prognosis. Etiology is largely unclear, but high doses of steroids have been associated with this. Further studying of possible correlation is vital to improve outcomes in OLT population.

Case Description/Methods: A 48-year-old woman with decompensated alcoholic cirrhosis was transferred for liver transplant evaluation after presenting with jaundice, ascites, and a MELD score of 41. She underwent OLT and was discharged on prednisone 20 mg, tacrolimus 3 mg BID, and mycophenolic acid 360 mg. There was no history of calcium, vitamin D supplementation or warfarin use. Post-operative course was complicated by rapid liver enzymes elevation (AST 1267 units/L, ALT 704 units/L) following initially down-trending values (AST 100 units/L, ALT 153 units/L) in the week following transplant. A liver biopsy was performed and showed mild-moderate acute cellular rejection (Banff 5). Prednisone was increased from 20 mg to 60 mg daily, with subsequent improvement in liver enzymes. A month later, she developed ulcers on her legs. She had a wedge biopsy, demonstrating multiple calcified small vessels in the subcutis and fat necrosis, indicative of calciphylaxis. Her kidney functions, calcium, phosphorus, vitamin D, and parathyroid hormone levels were within normal range (Table1). Similarly, auto-immune workup, protein C and protein S screen were unremarkable. Her prednisone was tapered off, sodium thiosulfate infusions were started, and wound care follow-ups were arranged; resulting in marked improvement in the lesions.

Discussion: NUC is a rare fatal condition associated with several risk factors. In this case, the cause of NUC was uncertain, triggering a thorough literature review for clinical correlation with OLT population. Etiology is largely unclear and prognosis varied, but there was some association with steroids. Given the timing of the increase in steroid dose and the appearance of the lesions, steroids were thought to be the cause, and where tapered off subsequently resulting in improvement.

Figure: A: Subcutaneous nodules with ulceration and sloughing of overlying skin involving the posterior calve.
B: Healed lesions with residual scarring after 3 months of treatment with sodium thiosulfate, wound care, and hyperbaric oxygen.

Lab test	Lab value	Reference range
Creatinine	1.05 mg/dL	0.56-1.00 mg/dL
eGFR	63 mL/min	>59 mL/min/1.73
Blood Urea Nitrogen	20 mg/dL	8.0 - 25.0 mg/dL
Calcium	9.6 mg/dL	8.4 - 10.1 mg/dL
Phosphorus	4.2 mg/dL	3.0 - 4.3 mg/dL
Vitamin D	25 ng/mL	20 - 80 ng/mL
Parathyroid hormone level	21 pg/mL	15 - 65 pg/mL

Table: Table 1: Calcium Phosphate Metabolism “Calciphylaxis” Lab Values

Disclosures:

Aldanah Althwanay indicated no relevant financial relationships.

Sassan Sakiani indicated no relevant financial relationships.

Aldanah Althwanay, MD, Sassan Sakiani, MD. A0554 - Recognizing Deadly Skin Lesions in a Post Liver Transplant Patient: A Survival Story, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.