Scleroderma renal crisis as a presenting feature in the absence of skin involvement

A 56-year-old man was admitted to the nephrology unit with a short (6-week) history of severe hypertension that necessitated renal replacement therapy within 7 days after admission. Renal biopsy showed features of thrombotic microangiopathy in arterioles and small arteries with occluding thrombi. The skin was unremarkable at the time of admission. Progressive skin lesions with scleroderma, telangiectasia, sclerodactyly, and generalized cutaneous sclerosis developed within 4 weeks and the specific skin changes were found on skin biopsy. On admission antinuclear antibody titers were high (1:10, 240) with a nucleolar pattern, and PM-Scl antibodies (1:5, 120) were present. In the present case the diagnosis of scleroderma renal crisis was made in vivo by renal biopsy. Renal and skin biopsies documented that renal lesions may precede the clinically manifest skin lesions of progressive systemic sclerosis.