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PubMed · 8115902

Systemic calciphylaxis.

Abstract

Systemic calciphylaxis is a rare, poorly understood syndrome of progressive ischemic necrosis, usually associated with hyperparathyroidism. The combination of hyperparathyroidism, usually secondary or tertiary, and chronic renal failure seems to produce a particular biochemical environment conducive to the development of an unusual progressive form of ischemia. Early recognition and diagnosis, appropriate therapy including wound care, and expeditious parathyroidectomy often will halt the progression of the disease, minimize limb loss, and prevent subsequent sepsis and death.

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BibTeXRIS

R B Kent, R T Lyerly. 1994. Systemic calciphylaxis.. https://doi.org/10.1097/00007611-199402000-00029

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[Calciphylaxis].

HISTORY AND CLINICAL FINDINGS: Acute, zigzag-shaped livid skin markings developed on both thighs of a 55-year-old woman who had been on dialysis for 6 years. Within 7 days these areas increased in size to about 10 cm in diameter and contained central dry and painful necroses. On legs, lower arms and hands hard subcutaneous nodules were palpable with a diameter up to 3 mm. For many years the phosphate and parathormone levels, as well as alkaline phosphatase activity had been raised. The patient had often failed to follow treatment recommendations. TESTS: There were increased serum concentrations of calcium (2.8 mmol/l) and phosphate (1.78 mmol/l). The calcium phosphate ion product was 4.98 (mmol/l)2. Furthermore, there were raised levels of alkaline phosphatase (315 U/l) and parathormone (1076 ng/l, normal: 10-65). X-ray film of the hands showed soft tissue and arterial calcifications, while histological examination of a deep skin biopsy revealed calcium phosphate emboli of the main vessels. TREATMENT AND COURSE: Excision of the cutaneous necroses was followed by parathyroidectomy at which only three parathyroid glands were identified and removed. The parathormone level fell postoperatively, but rose again after 4 weeks. The fourth parathyroid gland was then found and removed, after which the parathormone level fell below measurable levels. The skin ulcers healed completely 4 weeks after the second operation.

Calciphylaxis

Hyperbaric oxygen therapy in calciphylaxis-induced skin necrosis in a peritoneal dialysis patient.

A 58-year-old white woman on continuous ambulatory peritoneal dialysis for 2 years developed calciphylaxis-induced necrotic skin lesions over both lower extremities. Despite subtotal parathyroidectomy and other conventional measures, skin lesions continued to worsen. Mapping of transcutaneous oxygen pressure showed markedly low values in involved areas. Skin ulcers completely healed after 38 sessions of hyperbaric oxygen therapy. The results in our case indicate that hyperbaric oxygen therapy may be useful in the treatment of skin ulcers secondary to calciphylaxis.

Calciphylaxis