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| CASE REPORT |
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| Year : 2012 | Volume
: 1
| Issue : 3 | Page : 166-168 |
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Panniculitis-polyarthritis-pancreatitis syndrome
Sanket K Mahajan, Swati C Aundhakar, Makarand B Mane
Department of Medicine, Krishna Institute of Medical Sciences University, Karad, Maharashtra, India
| Date of Web Publication | 15-Jan-2013 |
Correspondence Address:
Swati C Aundhakar
Prof. Aundhakar C. Swati, Saurabh Hospital 140, Budhwar Peth, Karad, Satara, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2278-0521.106089
Panniculitis and polyarthritis may rarely complicate a pancreatic disease; this association is defined as PPP-syndrome and is characterized by significant morbidity and mortality. Panniculitis is related to subcutaneous (liquefactive) fat necrosis with secondary skin changes due to the local activity of pancreatic enzymes released during different pancreatic diseases (inflammatory, acute and chronic, and neoplastic). The reported incidence of association of panniculitis and pancreatitis is 2-3% of all pancreatic diseases, while the complete PPP-syndrome is reported in very few cases. Here, we report a 49-year-old man with acute on chronic pancreatitis who presented to us with abdominal pain, arthritis, and multiple subcutaneous swellings which were present all over the body (generalized) unlike the commonly reported below-knee distribution, and this is what makes our case different. Biopsy from the subcutaneous swellings revealed fat necrosis and USG abdomen showed features of pancreatitis. Keywords: Generalized panniculitis, PPP-syndrome, rare
How to cite this article:
Mahajan SK, Aundhakar SC, Mane MB. Panniculitis-polyarthritis-pancreatitis syndrome. Saudi J Health Sci 2012;1:166-8 |
| Introduction | |  |
Panniculitis and polyarthritis may rarely complicate pancreatic disease; this association is defined as PPP-syndrome and is characterized by significant morbidity and mortality. The reported incidence of association of panniculitis and pancreatitis is 2-3% of all pancreatic diseases, while the complete PPP-syndrome is reported in very few cases. Less than 30 such cases have been reported in the literature. [1] Polyarthritis is preferentially symmetric with more frequent involvement of legs than arms and negativity of serum rheumatoid factor. Due to the absence of abdominal symptoms in most cases, delay in diagnosis is not unusual. [2]
| Case Report | | |
A 49-year-old male patient came with the complaints of pain in abdomen, mainly in epigastric region, radiating to back with abdominal distension and markedly reduced appetite since 5 days before admission. Pain was relieved after three episodes of vomiting which was greenish in color, non-projectile, containing food particles. Patient also complained of swelling over both lower limbs, which was tender with multiple joint pain involving almost all joints of the body. Patient also had multiple hyperpigmented nodules all over abdomen [Figure 1] and back and both the lower limbs, waxing and waning since 3 years. Patient was a diagnosed case of pancreatitis since 5 years. There was no history of fever or loose motions. Patient was a binge alcoholic for 15 years (country liquor, around 180 ml daily) and tobacco chewer for 10 years. On admission, the patient was pale and had a pulse rate of 110/min with BP 90/50 mmHg. He had bipedal pitting edema with gross signs of inflammation. On examination, he had ascites with guarding all over the abdomen with absent peristalsis. On detailed investigations, his reports showed the following:
Hb, 11.9 gm%; total leukocyte count (TLC), 51,000/cmm; P 90%, L 10%; platelets, 165,000/cmm; Prothrombin Time (International Normalised Ratio)), 1.0 Blood sugar level, 101 mg/dl; urea, 80 mg/dL; creatinine, 1.4 mg/dL; Na + , 120 mEq/L; K + , 3.2 mEq/L; serum amylase, more than 1500 IU; serum calcium, 6.5 mg/dL; urinary amylase, more than 2000; total bilirubin, 0.6 mg/dL; serum glutamate oxaloacetate transaminase (SGOT), 42 IU; serum glutamate pyruvate transaminase (SGPT), 14 IU; alkaline phosphatase, 116mg/dL; total proteins, 4.0 mg/dL; serum albumin, 2.5 mg/dL; erythrocyte sedimentation rate (ESR), 80 mm/h; C-reactive protein (CRP), positive with titer 0.4 mg/L; serum uric acid, 3.0 mg/dL (Hepatits B Antigen, Hepatitis C Antigen, VDRL, Rhematoid Factor: Negative).
Ultrasonography of abdomen and pelvis: Pancreas appeared bulky with peripancreatic fat collection with irregularity of surface and altered echogenicity (hypoechoic) suggestive of pancreatitis, gross ascites, and splenomegaly.
The dermatologist was consulted for the hyperpigmented skin lesions, which was diagnosed to be multiple extensively tender, deep-seated hyperpigmented resolving nodules with erythematous base suggestive of enzymatic panninculitis, and biopsies were taken for confirmation of diagnosis which showed pancreatic enzyme panniculitis.
The patient was treated with injectable Meropenem (1 g IV q 8 hourly) along with IV albumin, injectable octreotide, and parenteral nonsteroidal anti-inflammatory drugs (NSAIDs) along with parenteral nutrition. Patient's symptoms started reducing gradually and patient was gradually allowed oral feeds and eventually on put on oral pancreatic enzymes and oral NSAIDs along with other supportive care, and eventually he was discharged in a hemodynamically stable condition. On further follow-ups for the next 1 month, he was totally symptom-free.
| Discussion | | |
Many cases of pancreatitis have been reported. Although there are some patients with panniculitis and elevated serum lipase levels without pancreatic disease, [2] most patients with pancreatitis have acute or chronic pancreatitis [3] and some have pancreatic tumors or ischemic disease. [4],[5] The patient discussed here had a history of chronic pancreatitis with many acute flare-ups over a 5-year period. The typical patient with PPP-syndrome is a middle-aged male with heavy alcohol use. Panniculitis and polyarthritis may rarely complicate a pancreatic disease; this association is known as PPP-syndrome and is characterized by relevant morbidity and mortality.
Some common causes of panniculitis are trauma to skin, lymphoma, pancreatitis, cold temperature, infection, alpha-1 deficiency, etc., Pancreatic panniculitis occurs in less than 3% of patients with pancreatic disease. [6] Narvaez et al. attributed the arthritis to the effect of lipolytic pancreatic enzymes binding to adipose cell membranes. This initiates hydrolysis of triglycerides into free fatty acids, the release of which leads to acute arthritis. [4] Elevated levels of pancreatic enzymes can induce fat necrosis in the joint and adjacent periarticular tissues and leads to arthritis. [7] Polyarthritis is preferentially symmetric with more frequent involvement of ankles, but can also involve the small joints of the hands, wrist, and feet, with the negativity of serum rheumatoid factor.
The exact mechanism of fat necrosis associated with pancreatic disease is still debatable and many mechanisms have been suggested. The most popular theory consists of the involvement of the pancreatic enzymes lipase, phosphorylase, trypsin, and amylase. Trypsin may increase the permeability of the microcirculation, allowing lipase and amylase to enter the fat lobules and hydrolyze the neutral fat to form glycerol and free fatty acids. This leads to the associated fat necrosis and inflammation. [6],[8],[9] Another theory postulates that panniculitis is related to subcutaneous (liquefactive) fat necrosis with secondary skin changes due to the local activity of pancreatic enzymes released during different pancreatic diseases (inflammatory, acute and chronic, and neoplastic). Clinically, lesions present as tender or non-tender, edematous, erythematous, or red-brown nodules that may spontaneously ulcerate and drain an oily brown, sterile, and viscous substance. Discharge from the lesions is a common feature of pancreatic panniculitis. [6],[8],[9] This is a result of liquefactive necrosis of adipocytes and, with the exception of alpha 1-antitrypsin deficiency, is unique to pancreatic panniculitis. Lesions are usually present in distal parts of lower extremities and are commonly misdiagnosed as erythema nodosum (EN). [6],[9] A distinguishing feature is that the lesions of pancreatic panniculitis may migrate proximally over the arms and trunk. [4],[9] The occurrence of lesions of panniculitis over the abdomen is very rare, which is unique in our case, making it the first case in the medical literature.
Thus, we hereby conclude that a very high index of suspicion is required to diagnose PPP-syndrome in a patient getting admitted for suspected acute pancreatitis with an incidental finding of skin lesions in the form of panniculitis (the treatment of which is mainly supportive along with the treatment of the underlying pancreatic disease) associated with polyarthritis so that the treatment can be appropriate for good outcome of the patient in terms of morbidity and mortality.
| References | | |
| 1. | Opsomer D, Suttels V, De Keyser K, Spanoghe G, Goeteyn V, Deprez S, et al. Pancreatic disease, Panniculitis and Polyarthritis (PPP) Syndrome: A case report and review of the literature. Belg J Med Oncol 2011;5:154-8. 
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| 2. | Forstrom TL, Winkelmann RK. Acute, generalized panniculitis with amylase and lipase in skin. Arch Dermatol 1975;111:497-502.
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| 3. | Francombe J, Kingsnorth AN, Tunn E. Panniculitis, arthritis and pancreatitis. Br J Rheumatol 1995;34:680-3.
[PUBMED] |
| 4. | Narvaez J, Bianchi MM, Santo P, de la Fuente D, Rios-Rodriguez V, Bolao F, et al. Pancreatitis, panniculitis, and polyarthritis. Semin Arthritis Rheum 2010;39:417-23.
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| 5. | Smukler NM, Schumacher HR, Pascual E, Brown S, Ryan WE, Sadeghian MR. Synovial fat necrosis associated with ischemic pancreatic disease. Arthritis Rheum 1979;22:547-53.
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| 6. | Garcia-Romero D, Vanaclocha R Pancreatic panniculitis. Dermatol Clin 2008;26:465-70.
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| 7. | Mustafa KN, Hadidy A, Shoumaf M, Razzuki SA. Polyarthritis with chondronecrosis associated with osteonecrosis, panniculitis and pancreatitis. Rheumatol Int 2010;30:1239-42.
[PUBMED] |
| 8. | Phelps RG, Shoji T. Update on panniculitis. Mt Sinai J Med 2001;68:262-7.
[PUBMED] |
| 9. | Dahl PR, Su WP, Cullimore KC, Dicken CH. Pancreatic panniculitis. J Am Acad Dermatol 1995;33:413-7.
[PUBMED] |
[Figure 1]
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