RESUMO
Wegeners granulomatosis is a rare puzzling disease generally characterized by necrotizing granulomatous lesions in the lungs and upper respiratory tract generalized necrotising vasculitis and acute glomerulitis Pathologically tehre is focal necrotizing vasculitis involving the small arteries and veins of the respiratory tract as well as those of the kidney spleen heart intestines liver mucous membranes and skin. In this rport we wish to stress that the cutaneous manifestations are often a prominent feature of wegeners granulomatosis and occasionally are the first sign of the desease. The basis for this report is II8 patients with unequivocal wegener granulomatosis screened from the word literature. The authors cheched al the reported cases of wegeners granulomatosis as well as the conditions frequently confused with it such as periarteritis nodosa lethal medline granuloma the stevens johson syndrome and several others. With but few exceptions patientes were included in this series only if an apparent diagnosis of wegeners granulomatosis was confirmed by gross autopsy examination or by microscopy biopsy examination using the widely accepted pathologic criteria of Godman and Churg (2). If there were any doubt about the diagnosis in any patient in any series or if the pathologic evidence were only presumptive that patient wasexcluded from this study. Our object was to review as many patients as possiblewith clear cut wegeners granulomatosis in order to study the extent of the cutaneos involvement in this disease. In addition to illustrate some of the problems inherent in distinguishing wegener granulomatosis from other conditions characterized by necrotizing angiitis with granuloma we have included five cases form our personal experience. All of these had mucosal lesions and four of the five had atypical pneumonias. Autopsy examination of the four who have died indicated that there had authentic wegeners granulomatosis while the evidence in the other two patients suggests their condition possibily could be due to this entity yet there are many atypical features that cast doubt on the diagnosis.