Juvenile systemic granulomatosis manifesting as premature aging syndrome and renal failure

We describe a case of juvenile systemic granulomatosis in a 22-year-old woman. The rash consisted of purple papules and first appeared at the age of one year. She had persistent symmetrical painless boggy tenosynovitis with minimal roentgenographic changes and chronic granulomatous symptoms. Uveitis resulted in visual impairment. She also had granulomatous changes in her vessels. Renal impairment developed; however, neither renal artery stenosis nor hypercalcemia was found. Clinical features included the development of premature aging with alopecia, which differed from the previously reported progeria syndrome. Poikiloderma may cause a prematurely aged appearance. Our report expands the clinical spectrum of systemic granulomatosis to include the development of premature aging with alopecia.