Pancreatitis, panniculitis and polyarthritis (PPP) syndrome.

A young male presented with intermittent high-grade fever, asymmetric polyarthritis and erythematous, tender nodules over left shin for 2 months duration. He had a history of alcohol dependence with multiple episodes of acute pancreatitis. With polyarthritis progressing relentlessly, unresponsive to non-steroidal anti-inflammatory drugs and steroids, a provisional diagnosis of sarcoidosis was considered. Indeed, he was treated with azathioprine and rituximab with no effect. Biopsy of the skin nodule revealed subcutaneous fat necrosis, foam cells, deposition of eosinophilic amorphous material and calcification. Synovial fluid aspiration from the arthritic knee obtained purulent but surprisingly culture-negative material, rich in triglycerides. Abdominal CT confirmed chronic pancreatitis. Final diagnosis of pancreatitis, panniculitis and polyarthritis (PPP) syndrome was made. He underwent surgical pancreatic ductal drainage leading to complete remission of symptoms. PPP syndrome triad occurs due to leakage of pancreatic enzymes into systemic circulation and subsequent fat necrosis. Surgical drainage of pancreatic duct is often curative.

Case report on the radiographic features of epipericardial fat necrosis in a patient presenting with acute chest pain.

Epipericardial fat necrosis (EFN) is a rare and benign condition. The three cardinal features of EFN are acute pleuritic chest pain, radiological features of an encapsulated fatty lesion within the epipericardial fat and the presence of inflammation within the surrounding pericardium. The exact aetiology is still unknown, and there are no known risk factors. The condition appears to be a transient process with no associated long-term complications. In this case study, we demonstrate the multitude of radiological features associated with this condition. A 29-year-old Caucasian female presented with acute pleuritic chest pain. There were no acute changes on her blood work, electrocardiogram (ECG) or echocardiogram. The chest radiograph showed an opacity projected within the anterior mediastinum. Further imaging, with computed tomography (CT) chest with contrast, was performed to define the characteristics of the opacity. This confirmed an encapsulated, mixed fat, soft tissue density in the left pericardiac region deemed to represent EFN. Follow-up magnetic resonance (MR) cardiac imaging at 1, 4 and 12 months demonstrated the self-resolving characteristics of this condition. EFN is a rare differential diagnosis to consider in patients presenting with acute pleuritic chest pain when laboratory tests are normal and there are no acute ECG findings. EFN should be excluded by imaging with CT or MRI of the heart. We have demonstrated through follow-up MR imaging the progression and resolution of EFN over 12 months.

Epipericardial fat necrosis in chest CT and MRI: a case report of an unusual cause of chest pain associated with the initial diagnosis of undifferentiated connective tissue disease.

BACKGROUND: Epipericardial fat necrosis (EFN) is a benign and self-limited condition of unknown cause with a good prognosis, usually affecting otherwise healthy patients. Clinically, it presents with severe acute left pleuritic chest pain, often leading the patient to the Emergency Room (ER). CASE PRESENTATION: A 23-year-old male, smoker (5 pack-years), was evaluated in the ER due to left pleuritic chest pain, worsening with deep breathing and Valsalva maneuver. It was not associated with trauma and did not present other symptoms. The physical examination was unremarkable. The arterial blood gases while breathing room air and the laboratory tests, including D-dimers and high-sensitivity cardiac Troponin T, were normal. The chest radiograph, electrocardiogram, and transthoracic echocardiogram showed no abnormalities. A computed tomography (CT) pulmonary angiogram showed no signs of pulmonary embolism but depicted at the left cardiophrenic angle a focal 3 cm ovoid-shaped fat lesion with stranding and thin soft tissue margins, consistent with necrosis of the epicardial fat, which was confirmed by magnetic resonance (MRI) of the chest. The patient was medicated with ibuprofen and pantoprazole, with clinical improvement in four weeks. At a two-month follow-up, he was asymptomatic and presented radiologic resolution of the inflammatory changes of the epicardial fat of the left cardiophrenic angle on chest CT. Laboratory tests revealed positive antinuclear antibodies, positive anti-RNP antibody, and positive lupus anticoagulant. The patient complained of biphasic Raynaud's phenomenon initiated five years ago, and a diagnosis of undifferentiated connective tissue disease (UCTD) was made. CONCLUSIONS: This case report highlights the diagnosis of EFN as a rare and frequently unknown clinical condition, which should be considered in the differential diagnosis of acute chest pain. It can mimic emergent conditions such as pulmonary embolism, acute coronary syndrome, or acute pericarditis. The diagnosis is confirmed by CT of the thorax or MRI. The treatment is supportive and usually includes non-steroidal anti-inflammatory drugs. The association of EFN with UCTD has not been previously described in the medical literature.

Subcutaneous fat necrosis of the newborn: A retrospective study of 32 infants and care algorithm.

OBJECTIVE: To describe the clinical and laboratory outcomes of infants with subcutaneous fat necrosis of the newborn (SCFN) and propose a care algorithm. METHODS: This single-center, retrospective study of infants diagnosed with SCFN at Ann & Robert H. Lurie Children's Hospital of Chicago from 2009 to 2019. RESULTS: Of 32 infants who met inclusion criteria, most were born full-term (84%), born via cesarean section (58%), had normal weight for gestational age (69%), and experienced delivery complications (53%). Twenty-nine infants (91%) had calcium drawn, and all had hypercalcemia. Three infants developed clinical symptoms of hypercalcemia, two required hospital admission, two developed nephrocalcinosis, and one developed acute kidney injury. The majority of infants (62%) had a peak ionized calcium between 1.5 and 1.6 mmol/L. No infants with peak ionized calcium less than 1.5 mmol/L developed complications of hypercalcemia. Most patients were diagnosed with hypercalcemia (86%) and demonstrated peak ionized calcium levels (59%) within the first 28 days of life. No patients developed hypercalcemia after 3 months of age. CONCLUSION: Hypercalcemia occurred in 100% of infants who had laboratory monitoring. We recommend obtaining an initial ionized calcium level when SCFN is suspected, and monitoring for the first 3 months of life if hypercalcemia has not been detected. In patients with asymptomatic hypercalcemia less than 1.5 mmol/L, there appears to be low likelihood of related complications. For symptomatic, markedly elevated (>1.6 mmol/L), or persistently elevated levels (>6 months) we suggest coordinated care with endocrinology or nephrology, consider hospitalization, and urinary system ultrasound.

Self-induced panniculitis in adolescence: Three cases and review of the literature.

Self-induced trauma is a rare cause of panniculitis in adolescents and may be associated with undiagnosed underlying psychological conditions. The condition often poses a diagnostic challenge as patients usually present with non-specific signs and symptoms, and often a concealed history of self-induced trauma. Here, we present three adolescent patients with self-induced traumatic panniculitis. All three patients were repeatedly admitted with non-specific cutaneous lesions as well as multiple somatic complaints. After extensive evaluation to exclude organic causes, psychiatric assessment eventually revealed underlying psychosomatic disorders as a cause of the self-induced trauma.

A woman with recurrent umbilical bleeding: a case report.

BACKGROUND: Umbilical discharge in an adult is rare and generates broad diagnostic considerations. Umbilical anatomy is variable owing to congenital abnormalities and acquired pathology such as umbilical hernias. The umbilicus can be a site of primary or metastatic malignancy or endometriosis. CASE PRESENTATION: A 40-year-old white American woman came to the clinic with a 2-day history of spontaneous umbilical bleeding. She reported periumbilical pain associated with nausea and emesis. There were no visible skin abnormalities, but deep palpation of the abdomen produced a thin, watery, serosanguineous fluid from the umbilicus. She experienced a similar episode of umbilical bleeding 5 years prior without clear cause. Laboratory workup was notable for mildly elevated C-reactive protein . Computed tomography imaging revealed a fat-containing umbilical hernia with fat necrosis, necessitating complete surgical resection of the umbilicus. CONCLUSIONS: Umbilical hernia with fat necrosis is a rare condition that should be considered in adults with umbilical discharge. Additional diagnostic considerations in adults with spontaneous umbilical bleeding/discharge include embryonal remnants, omphalitis, and metastasis. If the cause is not readily apparent on physical exam, imaging with computed tomography should be considered to assess for hernia and embryonal anomalies.

Epipericardial Fat Necrosis: A Retrospective Analysis in Japan.

Objective Epipericardial fat necrosis (EFN) has been considered to be a rare cause of acute chest pain, and especially important for emergency physicians. Chest computed tomography (CT) is often used for the diagnosis of EFN after excluding life-threatening states, such as acute coronary syndrome and pulmonary embolism. While the proportion of EFN patients who underwent chest CT in emergency departments is being clarified, little is still known about other departments in Japan. To investigate the proportion of EFN patients who underwent chest CT for acute chest pain in various departments. Methods Chest CT performed from January 2015 to July 2020 in Asahikawa Medical University Hospital in Japan was retrospectively analyzed in this study. All images were reviewed by two radiologists. Results There were 373 outpatients identified by a search using the word 'chest pain' who underwent chest CT. Eight patients satisfying the imaging criteria were diagnosed with EFN. The proportions of patients diagnosed with EFN were 10.7%, 4.8%, 2.8%, 0.9% and 0% in the departments of general medicine, cardiovascular surgery, emergency medicine, cardiovascular internal medicine and respiratory medicine, respectively. Only 12.5% of the patients were correctly diagnosed with EFN, and the other patients were treated for musculoskeletal symptoms, acute pericarditis or hypochondriasis. Conclusion EFN is not rare and is often overlooked in various departments. All physicians as well as emergency physicians should consider the possibility of EFN as the cause of pleuritic chest pain.

Subcutaneous and Intraosseous Fat Necrosis Associated with Chronic Pancreatitis.

Background: Extra-abdominal manifestations of fat necrosis, like subcutaneous fat necrosis, polyarthritis, and polyserositis may appear with an occurrence rate of about 0.8%, wherein intraosseous fat necrosis is a more rare complication of pancreatitis, with few reports in English literature. Case report: A 34-year-old male with a 15-year-history of alcohol abuse was hospitalized several times in the last few years because of attacks of relapsed chronic pancreatitis. After the last attack, pancreatitis came in a stable state ("burned out") with no symptoms and signs of the disease. The patient had been free of symptoms for 28 months since the last admission when he came with sub-febrile temperature, huge pain, swelling, and erythema in the area of the left lateral malleolar region with propagation in the foot. Blood biochemistry was normal. Conventional radiography showed multiple sites of osteolysis in the left calcaneus. Images on multislice computed tomography (MSCT) with 3D reconstruction revealed hypodense focuses that corresponded to osteonecrosis areas and bone marrow edema in the left calcaneus. Conclusions: The possibility of intraosseous fat necrosis should be considered in situations of unexplained polyarthritis or panniculitis, particularly in individuals with alcohol abuse or pancreatic disease.