Unilateral eyelid edema and mucosal involvement as the first presentations of Wegener granulomatosis

BACKGROUND: Wegener granulomatosis or granulomatosis with polyangiitis is a pauci-immune small vessel vasculitis which is usually associated with anti-neutrophil cytoplasmic antibodies (ANCA) mainly in old men. This small vessel vasculitis is usually characterized by necrotizing granulomatous inflammation with multiorgan involvement. Kidneys could be involved as the main and life-threatening condition in Wegener granulomatosis. Oral or ocular lesions may occur as the first and uncommon presentations before internal organ involvement in these patients and could be misdiagnosed with other diseases. CASE PRESENTATION: We present a 24-year-old man with erosions and ulcerations on palatal mucosa and a strawberry-like gingival hypertrophy associated with nasal congestion and epistaxis which two stated months ago. Also he had an episode of unilateral blepharitis and upper eyelid edema five months ago. Mucosal biopsy showed perivascular infiltrations of mainly neutrophils, some eosinophils and rare giant cells. He had elevated level of proteinase 3–ANCA or C-ANCA with microscopic hematuria without significant kidney involvement in kidney biopsy. Mucosal lesions and hematuria improved after two months of treatment with oral prednisolone. CONCLUSION: Unilateral eyelid edema and mucosal erosions in a young man could be the uncommon presentations of Wegener granulomatosis.