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The vascular calcification-cutaneous necrosis syndrome
P R Dahl1, R K Winkelmann, S M Connolly
1Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Vascular calcification-cutaneous necrosis syndrome, a rare condition causing skin ulcers due to abnormal calcium metabolism, can be treated. Successful treatment in one patient involved parathyroidectomy and kidney transplantation.
Area of Science:
- Dermatology
- Nephrology
- Vascular Biology
Background:
- Cutaneous vascular calcification is rare, unlike medial calcification of larger arteries in chronic kidney failure.
- This study focuses on the rare vascular calcification-cutaneous necrosis syndrome.
Observation:
- Three patients presented with ischemic necrotic ulcers and underlying cutaneous vascular calcification.
- Abnormal calcium metabolism, stemming from chronic kidney failure or excessive vitamin D intake, was the clinical setting.
- Patients exhibited tender livedoid nodules and ulcerative plaques on the thighs and legs.
Findings:
- Histologic examination confirmed vascular calcification in all patients.
- The syndrome typically presents a chronic course with significant morbidity and mortality.
- Subtotal parathyroidectomy and kidney transplantation led to complete resolution in one patient.
Implications:
- The vascular calcification-cutaneous necrosis syndrome has unique clinical and histopathologic features.
- The pathogenesis is likely multifactorial, involving complex interactions.
- While skin lesion treatment is primarily supportive, underlying metabolic correction may offer resolution.
Background:
Although medial calcification of larger elastic arteries in 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 calcification-cutaneous necrosis syndrome and review the cause, clinical and pathologic features, and treatment of this syndrome.
Methods:
We describe three patients with ischemic necrotic ulcers and underlying cutaneous vascular calcification. The clinical setting was abnormal calcium metabolism 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 either chronic kidney failure or excessive vitamin D intake. Histologic study demonstrated vascular calcification. Although this syndrome usually has a chronic course with significant morbidity and mortality, subtotal parathyroidectomy followed by kidney transplantation resulted in 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 multifactorial. Treatment for the skin lesions is largely supportive.
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