Eosinophilic granulomatosis with polyangiitis and oral lesions: an atypical first sign of the disease. A literature review

Abstract

Aim Eosinophilic granulomatosis with polyangiitis (EGPA, also known as Churg-Strauss Syndrome) belongs to the group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) together with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis). Ear, nose and throat (ENT) manifestations in AAV are relatively common and in some cases the first sign of the disease; in contrast, oral lesions are less frequent and described in only a very limited number of case reports of EGPA. Although not curable, AAV can be successfully managed with immunosuppressive drugs and other novel agents, highlighting the importance of early diagnosis.  Methods Herein, we reviewed the results of a literature search for descriptions of oral lesions in EGPA. A literature review was conducted, consisting of a literature search, pragmatic searches of web sources and snowballing. Results The few cases reported document that oral lesions can occasionally be the first sign, albeit atypical, of the disease.  Conclusions Healthcare professionals who examine the oral cavity, and especially otolaryngologists and dentists, should always consider EGPA in the differential diagnosis of oral lesions to avoid delayed diagnosis and, most importantly, the potentially fatal consequences of the vasculitic stage of the disorder.