title: Limited Wegener's disease presenting as pharyngolaryngeal tumor.
abstract: Wegener's disease (WD) which is mostly a systemic illness rarely presents as isolated, monoorganic, limited disease. Limited pharyngolaryngeal WD is thus a very rare occurrence. We report the case of a 29 years old man who developed a pharyngolaryngeal tumor with clinically benign evolution, histologically showing granulomatous inflammation and small vessel vasculitis, with no signs of: tuberculosis, sarcoidosis, fungal disease, Hodgkin's disease or foreign body aspiration. p-ANCA's were positive. He was considered a limited form of WD and treated with moderate doses of corticoids and cotrimoxazole. One month later, the lesion diminished significantly. The finding of a pharyngolaryngeal tumor with granulomatous inflammation and vasculitis, in the context of p-ANCA positivity and without any evidence for another systemic granulomatous disease, suggested the diagnosis of limited WD. The response to treatment favoured this presumption. Limited pharyngolaryngeal WD is a rare disease, with a potential for life-threatening (even fatal) complications. It should be recognized early and treated promptly. Remissions can be achieved (even without the use of cyclophosphamide).
authors.affiliation: Colentina Clinical Hospital, Clinic of Internal Medicine, Bucharest, Romania.
authors.forename: S
authors.initials: S
authors.lastname: Tănăseanu
authors.forename: V
authors.initials: V
authors.lastname: Pompilian
authors.forename: Cristina
authors.initials: C
authors.lastname: Tănăseanu
authors.forename: Camelia
authors.initials: C
authors.lastname: Badea
authors.forename: Florica
authors.initials: F
authors.lastname: Stăniceanu
authors.forename: S
authors.initials: S
authors.lastname: Zurac
authors.forename: C
authors.initials: C
authors.lastname: Popescu
authors.forename: A
authors.initials: A
authors.lastname: Bacalbaşa
mesh_terms: D000328:Adult; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D007822:Laryngeal Neoplasms / Q000209:etiology*; D008297:Male; D010610:Pharyngeal Neoplasms / Q000209:etiology*