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Systemic calciphylaxis presenting as a painful, proximal myopathy
C L Edelstein1, M K Wickham, P A Kirby
1Department of Internal Medicine, University of Stellenbosch, Tygerberg, Cape Province, South Africa.
Postgraduate Medical Journal
|March 1, 1992
Summary
Systemic calciphylaxis can cause painful muscle weakness in kidney transplant patients, even before skin lesions appear. Consider this condition in patients with proximal myopathy, especially those on dialysis or post-transplant.
Area of Science:
- Nephrology
- Pathology
- Dermatology
Background:
- Systemic calciphylaxis is a rare but severe condition often associated with end-stage renal disease.
- It typically manifests as painful skin necrosis and soft tissue calcification.
- Proximal myopathy is not a commonly recognized initial symptom.
Observation:
- A renal transplant recipient presented with severe, painful proximal myopathy.
- The myopathy was the presenting symptom, preceding any skin or soft tissue necrosis.
- Diagnostic workup confirmed systemic calciphylaxis as the underlying cause.
Findings:
- The case highlights that painful proximal myopathy can be an early, and sometimes the sole, manifestation of systemic calciphylaxis.
- This challenges the traditional understanding of calciphylaxis presentation, which emphasizes cutaneous findings.
- Biopsy and imaging confirmed widespread vascular calcification and ischemic myopathy.
Implications:
- Clinicians should consider systemic calciphylaxis in dialysis and renal transplant patients with unexplained painful proximal myopathy.
- Early recognition, even without skin lesions, may allow for timely intervention and potentially improve outcomes.
- This case expands the differential diagnosis for myopathy in the renal patient population.