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  • Title: [Calcyphylaxis in a patient with end-stage renal disease in the course of systemic lupus erythematosus, diagnostic problems--case report].
    Author: Gebska E, Vanaga-Besser S, Zajeck W, Matuszewska G, Jarzab J.
    Journal: Pol Merkur Lekarski; 2009 Aug; 27(158):123-8. PubMed ID: 19856878.
    Abstract:
    Calcyphylaxis consists in vascular calcium deposition and skin necrosis, which appears in the final stage of renal failure. Advanced forms of systemic connective tissue disease such as lupus erythematosus, especially with concomitant vasculitis, leading to renal failure and uremia may be conducive to calcyphylaxis. We are presenting a case of a 50-year-old male requiring chronic peritoneal dialysis since the age of 43, due to end-stage renal disease resulting from chronic glomeluronephritis. The patient presented to our Dermatology Department with large and painful coalescent cutaneous lesions on both lower extremities, fingertips and lips. Both clinical picture and disease history indicated systemic vasculitis that was previously unrecognized. Additional examinations revealed the following markers of inflammation: leukocytosis (11 G/I) with normal level of eosinophils, CRP > 0.6 mg/I, ESR 120 mm/h, and IgG 20 g/l, without any source of infection. Indirect immunofluorescence showed the absence of antineutrophil cytoplasmic antibodies (ANCA) on polynuclear leucocytes. A distinct fluorescence pattern observed in liver sinusoids was suggestive of the presence of some form of anti-granulocyte antibodies. Anti-nuclear antibodies (ANA) were detected in serum (1:1000) with homogenous and microgranular fluorescence pattern. Anti-extractable nuclear antigen panel (ENA), C3/C4 levels, anti-smooth muscle, anti-dsDNA and anti-glomerular basement membrane (GBM) antibodies were within normal limits. Lupus band test revealed IgA and IgG deposits at the dermis-epidermis junction. Histopathological examination of the skin showed calcification complicated by small vessel thrombosis, however, it was non-specific for vasculitis. Our case represents an example of calcyphylaxis associated with a widespread medial vascular calcification in a patient with end stage renal disease secondary to systemic lupus erythematosus. Although renal dialysis itself is known to trigger calcyphylaxis, there have been few reports in literature suggesting that collagen vascular disease may also contribute to this process.
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