ISR Autumn Meeting 2018

The following abstract selected for presentation as an Oral Case was commended

Deniz Demirdal

TBA (18A152)

CONNECTING THE P’S IN PANCREATITIS, PANNICULITIS AND POLYARTHRITIS SYNDROME: A CASE REPORT AND LITERATURE REVIEW

Author(s)

D. Demirdal,*, L. Mulligan, A. Mohammad, K. O'Rourke

Department(s)/Institutions

Rheumatology Department, Midland Regional Hospital Tullamore, Tullamore, Ireland

Introduction

Pancreatitis, panniculitis and polyarthritis together make up a triad known as PPP syndrome. It is a rare extra-pancreatic manifestation of pancreatitis, comprising polyarthritis, intraosseous fat necrosis and panniculitis.

Aims/Background

With less than 100 cases published worldwide and often mild/absent abdominal symptoms, establishing a diagnosis can be difficult. We describe a case of PPP and review the literature.

Method

Case description and literature review.

Results

A 48 year old male, with a history of psoriasis and alcohol-induced pancreatitis, presented with oligoarthritis affecting small joints of hands and ankles. Imaging suggested osteomyelitis surrounding 4th MCP and PIP joints. Bone biopsy showed sterile osteomyelitis with profound intraosseous fat necrosis. A month later the patient re-presented with polyarthritis and dactylitis, associated with multiple tender subcutaneous nodules on lower limbs, confirmed as panniculitis on skin biopsy. The patient had 2 further similar presentations. Repeat bone biopsies confirmed sterile osteomyelitis with intraosseous fat necrosis. On the last occasion, the patient reported epigastric pain: high serum amylase levels led to diagnosis of acute pancreatitis. Review of case revealed that each prior presentation was preceded by an elevation of serum amylase levels yet with absence of abdominal symptoms. Therefore, this man fulfilled the criteria for PPP syndrome: panniculitis and polyarthritis, preceded each time by pancreatitis.

The current literature regarding PPP syndrome is limited to case reports. The typical presentation is a middle-aged male with a history of alcohol abuse. It is hypothesized that pancreatic pathology is the aetiological factor, however abdominal symptoms in most published case reports are not prominent. Pancreatic enzymes secrete into peripheral tissues, causing local lipolysis and subcutaneous tissue necrosis leading to panniculitis, polyarthritis and osteonecrosis. There is no evidence to inform us which cases of pancreatitis are associated with disseminated fat necrosis. Nor is there significant guidance on treatment options, however successful pancreatic treatment has led to resolution of accompanying panniculitis and polyarthritis. There are concerns that with acute flares of pancreatitis, necrosis of the bone may progress and cause spontaneous fractures.

Conclusions

Our case highlights the complexity of this rare diagnosis. Further research is needed to guide clinical diagnosis and treatment.