Discussion
Pancreatitis is a rare initial presentation of Wegener's granulomatosis (granulomatosis with angiitis). A recent case review from March 2011 identified only five published cases. None of these diagnoses were made on pancreatic histopathology. Two were made on renal biopsy, the others on parotid, nasal and post-mortem biopsies. Four out of five were cytoplasmic-ANCA positive. Although there is no pancreatic histology from this patient's original presentation, the evidence for a vasculitic aetiology is strong. The patient did not return to full health after initial discharge and no aetiology was found for the pancreatitis. Furthermore inflammatory markers were consistently raised. Later gastric mucosal and dermis histopathology confirmed an underlying systemic vasculitic process.
The prognosis of Wegener's pancreatitis is poor, with two such patients dying from complications of Wegener's. One of these patients died from sepsis secondary to anastamotic bowel leak, the other from haemorrhagic pneumonia. Untreated Wegener's has a significant mortality of 90% at two years. At the time of writing there are no reported cases of a Wegener's pancreatitis resulting in loss of pancreatic endocrine function.
The considerable delay of two years from initial presentation to diagnosis in this case is unusual, all other reported cases were diagnosed and treated within two months of presentation. However, once the diagnosis was made and treatment initiated for Wegener's granulomatosis, markers of disease activity rapidly improved and the ulcer began to heal. This case illustrates the necessity of considering rarer causes of common complications in patients with diabetes to ensure accurate diagnosis and appropriate treatment.
The case described has several atypical features: the ulcer was situated on the antero-medial aspect of his calf with no evidence of distal ulceration or venous insufficiency. There was no evidence of peripheral vascular disease or neuropathy; peripheral pulses were palpable and sensation was intact throughout. The time from development of diabetes to ulceration was short. This case serves as a reminder that in a patient with atypical features it is vital to consider the rarer causes of ulcers for accurate diagnosis and subsequent management.