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Calciphylaxis of the Upper Limbs-A Rare and Serious Disease with Multidisciplinary Treatments-A Case Series and
Mihaela Pertea1,2, Malek Benamor2,3, Andra-Irina Bulgaru-Iliescu1,2
1Department Plastic Surgery and Reconstructive, Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.
Insights
Calciphylaxis, a rare condition causing skin necrosis, is particularly lethal when affecting the upper limbs. Despite multidisciplinary care and surgery, prognosis remains poor, with no standardized treatment protocol established.
Area of Science:
- Dermatology
- Nephrology
- Vascular Surgery
Background:
- Calciphylaxis, or calcific uremic arteriolopathy, is a rare, lethal condition causing skin necrosis.
- It most commonly occurs in patients with end-stage renal disease but can affect others.
- Upper limb involvement is rare and progresses rapidly.
Abstract:
Background/Objectives: Calciphylaxis or calcific uremic arteriolopathy is a rare but highly lethal pathology that occurs most frequently in a uremic context, although it can also occur outside of this context. It is characterized by the appearance of necrotic skin lesions. Localization to the upper limbs is rare and has a similarly progressive evolution. Methods: We present a series of two cases-a male and a female-with calciphylaxis diagnoses (including biopsies) and with the patients undergoing dialysis for end-stage renal disease, both with infected and extensive necrotic lesions to the hands and fingers. Both cases required serial debridement treatments and amputations. A literature review was conducted using the precise search terms "calciphylaxis", "upper limb", "uremic calcific arteriolopathy", and "end-stage renal disease" from January 2010 to May 2024. Results: One of the two reported cases ended with the patient's death. The results of the literature review (comprising seven similar cases) confirmed the rarity of calciphylaxis lesion localization to the upper limbs and the high mortality rate among these patients despite administered treatments. No therapeutic protocol for these cases was confirmed. Conclusions: The treatment of calciphylaxis cases is multidisciplinary. Although surgical intervention is controversial, it is necessary in some cases, sometimes serially. Localization to the thoracic limbs has the same evolution and poor prognosis as other localizations. A standardized therapeutic protocol for these cases is still far from being established.
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