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Published on: May 31, 2016
The vascular calcification-cutaneous necrosis syndrome
P R Dahl1, R K Winkelmann, S M Connolly
1Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
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.
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