Successful endoscopic treatment of PPP syndrome

Aims PPP syndrome (pancreatic disease, panniculitis and polyarthritis) is a rare syndrome with high morbidity and mortality. Joint and skin lesions are caused by lipolysis and secondary inflamation changes. We present a case series of four patients with successful treatment.

Methods Our group consists of 4 patients (3 men, 1 woman, mean age 55 years) in whom PPP syndrome was diagnosed in our department during period of last 8 years. Pancreatic disease was chronic pancreatitis in three patients and acinar pancreatic cancer in one patient. Panniculitis and arthritis were chief complaints in all patients, laboratory test revealed high inflamatory markers and elevated serum amylase and lipase. In two patients there was no history of pancreatic disease prior to diagnosis of PPP syndrome.

Results Two patients with chronic pancreatitis and stenosis of pancreatic duct were treated with endoscopic stent placement. Patient with chronic pancreatitis and aparent pancreatomesenteric fistula underwent duodenopancreatectomy and patient with acinar pancreatic cancer had left-sided pancreatectomy with splenectomy. In all cases treatment resulted in immediate normalization of serum amylace and lipase and no new skin or articular lesions were observed during follow-up period of mean 53 months (14-101).

Conclusions PPP syndrome can be treated endoscopically in some patients with chronic pancreatitis. When endoscopic decompression of pancreatic ductal system is not successful and in cases with pancreatic neoplasia surgical treatment is indicated.

 

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Conflicts of interest

Authors do not have any conflict of interest to disclose.

Publication History

Article published online:
14 April 2023