PHOEBE TIN1, JULEE RAJESH1, KIRSTEN HEPBURN1,2
1Metro North Kidney Health Service, Royal Brisbane and Women's Hospital, Herston, Australia, 2School of Medicine, University of Queensland, Herston, Australia
Biography:
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Background: Commonly occurring in kidney failure patients, calciphylaxis is a calcific uremic arteriolopathy characterised by painful ulcerative lesions. We report an atypical presentation of calciphylaxis detected by imaging in the absence of typical skin lesions.
Case Report:
This is a case of a 36-year-old female, on haemodialysis for 3 years, for kidney failure secondary to X-linked Alport Syndrome, with a background of class III obesity and hyperparathyroidism. After embarking on weight loss of 15 kilograms, she presented with progressive tender subcutaneous nodules of bilateral lower limbs without any overlying skin changes. X-ray and CT demonstrated extensive subcutaneous calcifications. Calcium, phosphate and 25-hydroxy-vitamin D levels were normal, though her parathyroid hormone was 246 pg/ml despite cinacalcet 90mg daily. Nuclear medicine bone scan showed innumerable subcutaneous soft tissue nodules with foci of calcification throughout the bilateral lower limbs with associated mild to moderate osteoblastic activity, which could be calciphylaxis in the appropriate clinical context. Given the atypical presentation, incisional biopsy was performed and reviewed at the dermatopathology meeting and reported to be consistent with calciphylaxis. She was treated with sodium thiosulfate on haemodialysis, vitamin K1 10mg three times a week and underwent a parathyroidectomy 3 months later. Her recovery was complicated by hungry bone syndrome with high calcium requirements. Over 6 months there was clinical improvement in subcutaneous nodules and serial X-ray imaging demonstrated significant improvement in calcifications.
Conclusion:
Calciphylaxis commonly presents with its characteristic superficial skin lesions however, consideration should be made to atypical presentations especially in the setting of clinical risk factors. This case highlights the subcutaneous presentation of calciphylaxis and the possible use of serial X-rays to monitor progress of these lesions.