JUVENILE SYSTEMIC GRANULOMATOSIS MANIFESTING AS PREMATURE AGING SYNDROME AND RENAL-FAILURE

We describe a case of juvenile systemic granulomatosis in a 22-year-ol d woman. The rash consisted of purple papules and first appeared at th e age of one year. She had persistent symmetrical painless boggy tenos ynovitis with minimal roentgenographic changes and chronic granulomato us symptoms. Uveitis resulted in visual impairment. She also had granu lomatous changes in her vessels. Renal impairment developed; however, neither renal artery stenosis nor hypercalcemia was found. Clinical fe atures included the development of premature aging with alopecia, whic h differed from the previously reported progeria syndrome. Poikiloderm a may cause a prematurely aged appearance. Our report expands the clin ical spectrum of systemic granulomatosis to include the development of premature aging with alopecia.