A 58 year old man presented with skin manifestations of scleroderma wi
thout systemic involvement. Within few weeks of oral corticosteroids a
nd penicillamine therapy, rapidly progressive systemic sclerosis devel
oped. The deterioration manifested by skin lesions, diffuse interstiti
al restrictive lung disease, acute renal failure with normal blood pre
ssure values, and microangiopathic hemolytic anemia compatible with he
molytic uremic syndrome. Chronic renal failure developed and was treat
ed by dialysis, but the patient died due to sepsis. The course of rena
l involvement was normotensive; antihypertensive therapy was not presc
ribed even once.. The association of scleroderma renal crisis with nor
mal blood pressure is probably a rare and ominous combination.