Midfacial granuloma syndrome. A clinic and pathological report on four patients

Four cases of midfacial necrotizing lesions are reported. All patients were males with ages ranging from 25 to 76 years. The relationship between subjective symptoms and laboratory data prior to therapy (leukopenia, elevated ESR, increment of IgA and IgG), as well as between fever crisis with sweats and chills and the progression of the lesions were pathognomonic clinical signs for us. In all cases, paranasal sinus and nasopharynx were involved. Middle ear, eye and kidney involvement was present in 2 cases, and joints lesions only in one. Three patients died (2 of sepsis and one from hemorrhage) despite therapy. A pleomorphic cellular infiltrate with atypical lymphocytes and a tendency to angiocentricity was found in these cases. Such features and PAP positivity to beta and kappa chains led us to consider these lesions as an extranodal B-lymphocyte lymphoma-like. In the fourth case the histological picture was that of a necrotizing granuloma with clustered giant cells. This patient, treated only with prednisone, had a total remission of his symptomatology up to 11 years after the onset of the disease.