title: [Wegener's and Stewart's granulomatosis: a case report of Stewart's granulomatosis].
abstract: Wegener's and Stewart's diseases are two rare pathologies of unknown origin; both can cause disruptive facial lesions. Wegener's disease is a systemic pathology and generally involves the kidneys and lungs. Granulomatosis lesions are characterized by the necrotizing vasculitis involving the small vessels. From a diagnostic point of view the systemic features of Wegener's disease and the specific immunological findings (i.e. IgG autoantibodies vs. monocyte and neutrophil cytoplasm) make it possible to diagnose the disease precisely. Stewart's disease can be differentiated from Wegener's disease by the absence of any systemic lesions and the lack of necrotizing vasculitis. Pathogenesis of the disease is still unknown although immunohistochemical findings indicate that it is related to extranodal Tcell lymphomas. Stewart's disease is very aggressive with massive destruction of the midface tissues and prognosis is very poor (from a few months to 23 years). Surgery is generally ineffective in such disorders. The treatment for Wegener's disease includes the use of systemic steroids, immunosuppressive drugs and the sulfametoxazole-thrimethoprime association while radiotherapy associated with chemotherapy appears most effective in Stewart's disease. The authors describe a case of Stewart's disease prevalently involving the nasal cavities, ethmoid and paranasal sinuses. Diagnosis was made on the basis of immunohistochemical, histomorphological and immunological data. Treatment--based initially on systemic steroids with the association thrimethoprimsulfometoxazole--induced significant disease remission. Subsequent use of cytostatic drugs has made it possible to control the disease progression to date.
authors.affiliation: Dipartimento Regionale Testa Collo, Ospedale San Martino, Genova.
authors.forename: E
authors.initials: E
authors.lastname: Castello
authors.forename: G
authors.initials: G
authors.lastname: Caligo
authors.forename: J L
authors.initials: JL
authors.lastname: Ravetti
authors.forename: E A
authors.initials: EA
authors.lastname: Pallestrini
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D006099:Granuloma / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis*; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009297:Nasal Mucosa / Q000473:pathology; D010254:Paranasal Sinus Diseases / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D013256:Steroids
chemical_list: D000893:Anti-Inflammatory Agents; D013256:Steroids
vernacular_title: Le granulomatosi centrofacciali di Wegener e di Stewart: su un caso clinico di granuloma centrofacciale di Stewart.