WEGENERS GRANULOMATOSIS - CASE-REPORT AND LITERATURE-REVIEW

Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculiti s usually affecting the upper and lower respiratory tracts and kidneys . Any organ system can be affected by the pathologic process, which re mains an etiologic enigma. Limited forms of the disease are recognized in which few extrapulmonary and no renal lesions occur. Cutaneous man ifestations occur in 40% to 50% of patients with WG (1), Early diagnos is is imperative because treatment regimens reduce morbidity and morta lity in this potentially fatal disease. We report WG in a 10-year-old boy whose upper respiratory tract symptoms began at age 8 years. The c ase is illustrative of the difficulties that can be encountered in att empting to make this diagnosis. Skin is an easily accessible organ for biopsy, thus an increasing familiarity with the typical biopsy specim en findings within a consistent clinical setting may aid in earlier di agnosis of WG.