en Dermathology Dermathology case report case report <span style="font-size:12px;"><span style="font-family:times new roman;"><span style="color:#0000FF;"><strong><em>Background</em></strong><em>:</em></span><span style="color:#000000;"> 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.&nbsp; 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.</span><br> <span style="color:#0000FF;"><strong><em>Case presentation:</em></strong></span><span style="color:#000000;"> 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&ndash;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.</span><br> <span style="color:#0000FF;"><strong><em>Conclusion</em></strong><em>:</em></span><span style="color:#000000;"> Unilateral eyelid edema and mucosal erosions in a young man could be the uncommon presentations of Wegener granulomatosis.</span></span></span> Wegener’s granulomatosis, oral lesions, eyelid edema 343 346 http://caspjim.com/browse.php?a_code=A-10-658-2&slc_lang=en&sid=1 Rana Rafiei rafieirana2@gmail.com 100319475328460018113 100319475328460018113 Yes Skin Research Center, Dermatology Department, Guilan University of Medical Sciences, Razi Hospital, Narges Alizadeh narges.alizade7@gmail.com 100319475328460018114 100319475328460018114 No Skin Research Center, Dermatology Department, Guilan University of Medical Sciences, Razi Hospital, Kaveh Gharaeinejad gharaeek36@gmail.com 100319475328460018115 100319475328460018115 No Skin Research Center, Dermatology Department, Guilan University of Medical Sciences, Razi Hospital, Behnam Rafiee behnam2776@gmail.com 100319475328460018116 100319475328460018116 No Department of Pathology, NYUWinthrop Hospital, 222 Station Plaza, No. 620, Mineola, NY 11501, USA Sara Naji rad snajirad@gmail.com 100319475328460018117 100319475328460018117 No Department of Internal Medicine, Nassau University Medical Center, 2201 Hempstead Turnpike, East Meadow, NY 11554, USA