A 73 year patient presented with melena to our endoscopy unit. He was hemodynamically stable and the haemoglobin was decreased with 113 g/l. The remaining blood tests were normal. Upper endoscopy showed a large bleeding ulcer along the lesser curvature, macroscopically suspicious for malignancy. To control the bleeding and to rule out gastric cancer, the patient was referred to surgery. Two thirds of the stomach, a segment of the transverse colon with mesocolon had to be resected en bloc. Final histology revealed a chronic ventricular ulcer with a distinct lymphofollicular, lymphoplasmacellular and eosinophilic inflammation. The number of IgG4 plasma cells was increased (60 IgG4 positive cells per microscopic visual field, 70-80% IgG4 out of all IgGs). Storiform fibrosis and obliterating phlebitis were found not only within the ulcer base but also extensively in the adjacent soft tissue. Summarizing, our patient suffered from a rare form of a chronic gastric ulceration as a manifestation of IgG4-related disease. IgG4-related disease is an increasingly recognized condition presenting with specific pathological, serologic and clinical features. Its hallmark is the typical histopathological finding as in our case. Type 1 autoimmune pancreatitis and salivary gland disease are the two typical presentations, but IgG4-disease can affect each organ. Because of no further organ involvement, consecutive immunosuppressant treatment was not required. Seven months later gastroscopy showed no signs of recurrence. A gastric ulcer can be the first presentation of IgG4 related disease which needs to be considered as a differential diagnosis in refractory gastritis and cancer suspicious lesions.
