What is Pancreatic Panniculitis?
Pancreatic panniculitis is a rare skin condition first identified by Hans Chiari in 1883. It is a type of panniculitis (an inflammation of fatty tissue under the skin) related to damage in fat tissues, even when the damage is far from the pancreas. It is typically found in patients with chronic pancreatitis or pancreatic cancer, which includes acinar and islet cell carcinomas. Other types of pancreatic cancers, like ductal adenocarcinoma, neuroendocrine tumors, and intraductal papillary mucinous neoplasms, are also associated with pancreatic panniculitis.
If someone has pancreatic panniculitis, they may experience painful, red to purple, ulcerated lumps, usually seen on the arms and legs. These lumps may worsen to a point where they become liquid and die, causing spontaneous ulcers and a brown, sticky liquid to drain out. This skin condition is due to a large release of fat-breaking enzymes in the bloodstream, linked to the underlying pancreatic disease.
People with pancreatic panniculitis often feel ill overall with symptoms like fever and weight loss. Joint discomfort can also be prominent, and blood tests often show elevated levels of certain pancreatic enzymes, lipase and amylase. Interestingly, these symptoms can appear before the discovery of a pancreatic issue almost half of the time.
Since pancreatic panniculitis is linked to the unregulated release of pancreatic enzymes due to pancreatic cancers, it can also be considered a paraneoplastic syndrome. This is a term used for disorders triggered by the body’s response to certain cancers.
Also, it’s possible for arthritis to appear at the same time as pancreatic panniculitis, either before or along with the pancreatic disease. This occurrence has been termed as Pancreatic disease, Panniculitis, and Polyarthritis (PPP) syndrome.
What Causes Pancreatic Panniculitis?
Pancreatic panniculitis, a skin condition often seen in middle-aged men who regularly consume alcohol and have either temporary or long-term inflammation of the pancreas, called pancreatitis.
Surprisingly, while a type of pancreatic cancer called acinar cell carcinoma makes up about 1% of all primary pancreas cancers, it’s the cancer most commonly linked to pancreatic panniculitis. Roughly 16 out of 100 patients with acinar cell carcinoma also have signs of this skin condition.
This type of pancreatic cancer can also be associated with a syndrome known as the “PPP” syndrome, which is short for pancreatic, panniculitis, and polyarthritis.
Pancreatic panniculitis could sometimes be the first indication of other types of pancreatic cancer, which include ductal adenocarcinoma, intraductal mucinous cancer, and neuroendocrine tumors.
Other conditions like pancreatic pseudocysts (fluid-filled sacs in the pancreas), pancreas divisum (a birth defect where parts of the pancreas do not join together), post-traumatic pancreatitis, and hemophagocytic syndrome (a severe systemic inflammatory syndrome) have also been found in connection with this type of panniculitis. Further, post-ERCP pancreatitis, a condition that can occur after a type of X-ray test known as ERCP, can also present with pancreatic panniculitis.
There have also been cases where individuals have pancreatic panniculitis without any underlying pancreatic disease. In these cases, skin biopsies confirmed the characteristics seen in pancreatic panniculitis, even though there’s no detectable disease in the pancreas.
Risk Factors and Frequency for Pancreatic Panniculitis
Pancreatic panniculitis is a condition related to the pancreas. It affects around 2% to 3% of patients who have pancreas disorders. It is four times more likely to occur in men than women. The average age of patients diagnosed with this condition is typically around 60, and those who have issues with alcohol are found to be more prone to this disease. Patients suffering from both pancreatic panniculitis and cancer are generally older.
A recent analysis found that the number of pancreatitis cases, a disease of the pancreas, is increasing year on year worldwide. A significant reason behind the rise is an increase in alcohol consumption in many developing countries and Asian nations. Some regions in the United States are not experiencing a rise in pancreatitis because the numbers have reached a plateau, indicating a limit in new cases.
As the number of acute pancreatitis cases continues to grow worldwide, we may observe more cases of pancreatic panniculitis. But it’s tricky to confirm this because studies focused on acute pancreatitis usually don’t include data on pancreatic panniculitis. While there have been systematic reviews of past case studies on pancreatic panniculitis, it’s still challenging to identify its exact frequency and occurrence rate, as several causes exist for this condition.
Signs and Symptoms of Pancreatic Panniculitis
Pancreatic panniculitis often affects people in their 70s or older, particularly when it is related to pancreatic cancer. The main symptom is the appearance of tender nodules, or small bumps, generally on the lower part of the body. These small bumps may appear on the thighs, hips, buttocks, breasts, upper body, scalp, or around certain joints. Sometimes, these nodules come before the usual signs of pancreatitis or pancreatic cancer.
In some cases, the nodules may last for a long time and more severe symptoms like dark, central ulcers which drain dark, oily fluids may occur. When such symptoms appear, it often suggests that there may be an underlying pancreatic cancer.
These skin growths may not always cause pain. However, if the pain from the bumps becomes hard to manage, patients often seek medical help and undergo further tests.
In addition to the bumps on the skin, other symptoms can include moving joint pain along with the panniculitis, suggesting the PPP syndrome, a condition associated with pancreatitis or pancreatic cancer. In this case, patients can also experience fat necrosis inside the bone marrow and inflammation of the linings of the chest or abdominal cavities (polyserositis). Interestingly, in PPP syndrome, about one-third of patients may not have typical abdominal symptoms or may have very mild ones, while skin and joint issues could be more dominant.
Doctors, when examining such patients, should get a detailed patient history, specifically about alcohol use, any past surgeries or procedures, cancer history in the patient or the family, and any trauma.
Testing for Pancreatic Panniculitis
Pancreatic panniculitis is a condition that should be evaluated with a careful physical examination. This condition often presents as tender, firm, and reddish skin nodules that can appear even before signs of any pancreatic disease. Physicians need to explore potential causes and risk factors for a more serious condition like cancer, based on the patient’s medical history.
Various lab tests will be required, including a complete blood count, lipase, amylase, liver enzymes, bilirubin, and inflammatory markers. If the lipase level is quite high, it could indicate pancreatic cancer rather than inflammation of the pancreas or pancreatitis. Notably, lipase values greater than 4414 U/I show a higher likelihood for pancreatic cancer.
A CT scan of the abdomen and pelvis with contrast, which is a type of imaging using x-rays, can provide a detailed view of any inflammation or masses in the pancreas, or if the disease has spread to other parts of the body. This is very important in identifying if the skin condition, panniculitis, has an abdominal source.
In some cases, tumor markers may also be checked, such as CA 19-9. However, it’s crucial to note those markers don’t usually show up in certain subtypes of pancreatic cancer. Younger patients might have an elevated level of alpha-feto protein instead.
To confirm the diagnosis, a biopsy, or a tissue sample, is needed, which will typically show specific changes like dead fat cells and infiltrating white blood cells around the affected adipocytes (fat cells). This helps establish a definitive diagnosis of pancreatic panniculitis.
While not standard, MRI scans of the lower extremities have shown some unique features in cases of pancreatic panniculitis. Given the nature of the condition primarily involving the death of fat cells, an MRI typically shows minimal enhancement after the contrast is injected. However, there might be some enhancement at the peripheries due to local inflammation in the subcutaneous layer, which is the layer beneath the skin.
Treatment Options for Pancreatic Panniculitis
Pancreatic panniculitis is a skin condition linked to problems within the pancreas. The primary method to treat the skin symptoms of this condition is to address the issues going on within the pancreas itself.
For instance, for those suffering from acute pancreatitis, administering intravenous fluids and encouraging early feeding can help lower the risks of infection and reduce mortality rates. In turn, this approach also helps to lessen the problems of pancreatic panniculitis within just a few days.
Sometimes, pancreatic panniculitis is associated with tumors within the pancreas, often at an advanced stage. Although the appearance of skin lesions usually indicates advanced disease, early medical intervention can still provide some benefits. For instance, a specific cancer, known as pancreatic acinar cell carcinoma, has been successfully treated with a combination of cancer-fighting drugs, including folinic acid, fluorouracil, irinotecan, and oxaliplatin. After multiple treatments, the associated pancreatic panniculitis symptoms resolved.
While handling the underlying pancreatic disease can make the skin nodules disappear, they can sometimes leave behind hyperpigmented, or darkened, atrophic (thinned or weakened) scars. Using nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, and drugs that suppress the immune system solely to treat the skin condition is not generally an effective strategy. However, when the inflammation and nodules cause discomfort or pain, administering suitable pain relief can provide comfort to the patient.
What else can Pancreatic Panniculitis be?
When trying to diagnose pancreatic panniculitis, several other related conditions need to be considered. Panniculitides is a term used to describe a range of medical problems that involve inflammation in the layer of fat beneath your skin. Some of these can cause ulcers on the lower limbs.
The conditions that need to be checked when diagnosing pancreatic panniculitis include two specific forms of panniculitis that can bring about ulcers to the lower limbs:
- Erythema induratum (also known as nodular vasculitis): This is a type of panniculitis often seen in middle-aged women, leading to tender nodules on the lower legs, which might ulcerate. It is caused by an immune response to certain bacteria, such as Mycobacterium tuberculosis.
- Alpha-1-antitrypsin deficiency-induced panniculitis: This is a rare genetic disorder where there’s a deficiency in a protein called alpha-1-antitrypsin, resulting in extra protein buildup in the liver and lungs. This condition can cause panniculitis, leading to painful nodules on the lower legs that might ulcerate.
Erythema nodosum is another common form of panniculitis, but it’ll not be connected to pancreatitis and is rarely linked to pancreatic cancer. Necrolytic migratory erythema can also appear similar to pancreatic panniculitis, but it’s more like plaque formations rather than nodules.
The only way to definitively distinguish pancreatic panniculitis from other similar conditions is through a skin biopsy. But it’s also crucial to know about the presence of pancreatic disease when diagnosing pancreatic panniculitis considering other similarities in skin manifestations.
Other conditions to keep in mind when diagnosing pancreatic panniculitis include:
- Migratory thrombophlebitis
- Skin abscesses
- Lupus panniculitis
- Weber-Christian disease
- Arthropod bites
What to expect with Pancreatic Panniculitis
The outcome for patients with pancreatic panniculitis, a rare skin condition, greatly depends on the underlying cause. For instance, pancreatic acinar cell carcinoma, although rare, is the common form of pancreatic cancer associated with pancreatic panniculitis and unfortunately has a poor prognosis or outlook.
In the United States, approximately one in five patients develops severe acute pancreatitis, a sudden inflammation of the pancreas, which has a 20% mortality rate. This implies that out of every five patients, one might unfortunately not survive.
Possible Complications When Diagnosed with Pancreatic Panniculitis
If pancreatitis and cancer are not properly diagnosed early on, they can lead to complications when a condition known as pancreatic panniculitis occurs. This condition is usually an early sign of pancreatitis and pancreatic cancer. The complications associated are infections, pancreatic tissue death, abnormal connections between organs (fistulas), pancreatic inefficiency or failure, formation of cysts and pseudocysts, spread of disease to other regions of the body (metastasis), and heightened chance of death.
Another condition that can shorten lifespan to less than a year, called Lipase Hypersecretion Syndrome (LHS), is associated with a specific type of pancreatic cancer known as pancreatic acinar cell carcinoma. This condition, often spreading to the liver, causes increased release of lipase — an enzyme that helps break down fats in your body. Symptoms include pancreatic panniculitis, joint inflammation, and a rise in a type of white blood cell known as eosinophils. Pancreatic panniculitis can often be one of the initial signs of LHS, and it’s common for patients to have cancer that has spread to the liver.
In the case of a patient with rapidly deteriorating kidney function along with panniculitis, the autopsy revealed damage to multiple joints, renal membrane death resulting in abnormal kidney shapes, and death of fat tissue with calcification in the stomach lining and bone marrow.
Complications to be Aware of:
- Pancreatic infection
- Pancreatic tissue death
- Formation of abnormal connections between organs (fistulas)
- Pancreatic inefficiency or failure
- Formation of cysts and pseudocysts
- Spread of disease to other parts of the body (Metastasis)
- Increase in mortality
- Joint inflammation (in the case of Lipase Hypersecretion Syndrome)
- Increased eosinophils
- Damage to multiple joints
- Death of the renal membrane with abnormal kidney shapes
- Tissue death with calcifications in the stomach lining and bone marrow
Preventing Pancreatic Panniculitis
Strategies to prevent pancreatic panniculitis, a rare skin condition caused by diseases of the pancreas, mainly focus on preventing pancreatitis (inflammation of the pancreas) and reducing risk factors for cancer. It’s important to note that alcohol-related pancreatitis is a common cause of pancreatic panniculitis; thus, individuals at risk should abstain from alcohol. This involves alcohol counseling, where the focus is on changing the individual’s drinking habits, educating them on alcohol’s negative impacts on the pancreas, and considering their socioeconomic background. Stopping tobacco use can also lower the risk of pancreatic cancer.
Weight loss, dietary changes and avoiding alcohol can help patients with high blood triglyceride levels (a type of fat found in the blood) to lower the chances of developing pancreatitis as well.
Another way to lower the risk is through a surgical procedure known as cholecystectomy, which is the removal of the gallbladder to prevent the recurrence of pancreatitis caused by gallstones. To put it in perspective, 8% of individuals are at risk for a recurrence of acute pancreatitis within 40 days of leaving the hospital, but this risk jumps to 22% over 5 years if the gallbladder isn’t removed.
