[Unusual presentation forms of Wegener's disease]

Wegener's granulomatosis may appear with the classic granulomatous necrotizing vasculitis affecting both high and low airway and renal glomerule, and rarely small vessels in any other location. In this way we may find generalized and localized forms of the disease. Three atypical cases of the disease are reported. The first one as a limited form with only airway affection. The second one as an unusual case of otitis with progressive affection of I, II, V, VI, VII and VIII cranial nerves. The third one was a generalized case with sinusitis and orbital pseudotumor. Both first and second cases have been controlled for a very long period of time of 22 and 14 years respectively, and none of them has presented any other location of the disease.