Limited Wegener's disease presenting as pharyngolaryngeal tumor.
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).
Tănăseanu
S
S
Colentina Clinical Hospital, Clinic of Internal Medicine, Bucharest, Romania.
Pompilian
V
V
Tănăseanu
Cristina
C
Badea
Camelia
C
Stăniceanu
Florica
F
Zurac
S
S
Popescu
C
C
Bacalbaşa
A
A
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*