THE VASCULAR CALCIFICATION-CUTANEOUS NECROSIS SYNDROME

Background: Although medial calcification of larger elastic arteries i n chronic kidney failure and with advancing age is relatively common, calcification of the cutaneous vascular system is rare. Objective: Our purpose was to describe three patients with the vascular calcificatio n-cutaneous necrosis syndrome and review the cause, clinical and patho logic features, and treatment of this syndrome. Methods: We describe t hree patients with ischemic necrotic ulcers and underlying cutaneous v ascular calcification. The clinical setting was abnormal calcium metab olism from either chronic kidney failure or excessive vitamin D intake . Results: The clinical findings in all patients consisted of multiple tender livedoid nodules and ulcerative plaques on the thighs and legs , which developed in the setting of abnormal calcium metabolism from e ither chronic kidney failure or excessive vitamin D intake. Histologic study demonstrated vascular calcification. Although this syndrome usu ally has a chronic course with significant morbidity and mortality, su btotal parathyroidectomy followed by kidney transplantation resulted i n complete resolution in one of our patients. Conclusion: The clinical and histopathologic findings in the vascular calcification-cutaneous necrosis syndrome are unique. The pathogenesis is likely multifactoria l. Treatment for the skin lesions is largely supportive.