Idiopathic Lethal Granulomatous Ulceration of the Nose and Face*

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REPORTS have occasionally appeared in the literature concerning a group of unusual and ultimately fatal cases characterized by nonspecific granulomatous ulcerations of the upper respiratory tract and face. We shall review these clinical features and report 6 cases which we have observed at the Cleveland Clinic over a period of 15 years; 3 of these have terminated in death. In 2 of the 3 patients living the general course has been a gradual decline, characterized by symptoms strikingly similar in various stages to those cases in which the patients died. At the present time one of these patients is apparently well and entirely free from all symptoms. Another is entering the stage of early necrotizing lesions of the nasal septum, while the third is approaching the terminal phase.

In none of our cases has an etiologic agent been found which might possibly account for the severity and relentlessly progressive nature of this affliction despite exhaustive chemical, bacteriologic, and microscopic examinations. Thus we are unable to contribute further to previous investigations of the etiology of these peculiar and devastating lesions. Williams,1 in his excellent presentation, has summarized the possibilities, many of which we have considered from time to time. That the disorder is perplexing is evidenced by the diversity and multiplicity of diagnostic procedures and therapeutic approaches which have been utilized in an effort to bring the lesions under control.

The onomatology applied to this affliction has varied with different authors. It has been termed “lethal granulomatous ulceration of the midline. . .



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