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.

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.

[A Case Report of Subcutaneous Nodular Fat Necrosis Caused by Obstructive Pancreatitis Due to IPMN].

A 72-year-old man underwent hemodialysis because of end-stage renal failure. The patient often suffered acute pancreatitis and was diagnosed with main duct type intraductal papillary mucinous neoplasm(IPMN). Moreover, skin erythema with pain occurred and was treated as cellulitis using antibiotics; however, the skin lesions did not improve. Skin pathological findings indicated subcutaneous nodular fat necrosis due to pancreatitis. Subtotal stomach-preserving pancreaticoduodenectomy was performed, and the skin erythema with pain symptoms were relieved. The final diagnoses were ampullary carcinoma and intraductal papillary mucinous adenoma(IPMA). We experienced a rare case of subcutaneous nodular fat necrosis due to IPMN.

The Pancreatitis, Panniculitis, and Polyarthritis (PPP) Syndrome: Subcutaneous Nodular Fat Necrosis, a Cutaneous Marker of Pancreatic Neoplasia.

The pancreatitis, panniculitis, polyarthritis (PPP) syndrome is a rare skin, joint, and pancreatic disorder, also known as subcutaneous nodular fat necrosis. It results from obstruction of pancreatic ducts with direct secretion of pancreatic enzymes into the bloodstream, causing extra pancreatic fat necrosis with subcutaneous tissue and joint inflammation. It is usually a cutaneous sign of pancreatic cancer or pancreatitis. To our knowledge, this is the first case associated with a pancreatic pseudotumor. We describe a 59-year-old man initially presenting with numerous painful erythematous subcutaneous nodules due to a fibrous pancreatic pseudotumor and its extreme dermatologic disease, resulting in necrosis of the shin and foot so severe that an amputation of the lower leg above the knee was required, a complication not previously described, to our knowledge. We emphasize that PPP syndrome is a cutaneous marker of internal malignancy, most often of pancreatic cancer or pancreatitis, but in this case of a rare pancreatic pseudotumor.

Subcutaneous fat necrosis in an infant with hypoxic ischaemic encephalopathy stage 3: an uncommon association.

Subcutaneous fat necrosis (SCFN) is inflammation and necrosis of adipose tissue associated with hypoxia and hypothermia. It leads to various metabolic abnormalities, of which the most dreaded is hypercalcaemia. We report a case of a 7-week-old boy with history of birth asphyxia (hypoxic ischaemic encephalopathy stage 3) who presented to us with features suggestive of hypercalcaemia with bilateral nephrocalcinosis. On examination, there were multiple subcutaneous nodules on both arms. Evaluation revealed suppressed parathyroid activity along with low levels of 25(OH)vitamin D3 and elevated 1,25-dihydroxyvitamin D3 Skin biopsy confirmed the diagnosis of SCFN. He was managed with intravenous fluids, single dose of intravenous furosemide and oral prednisolone. Hypercalcaemia responded within 14 days of admission, prednisolone was tapered and stopped in a month. SCFN, in our case, can be attributed to the underlying perinatal asphyxia along with use of therapeutic hypothermia. Through this case, we wish to sensitise practicing neonatologists for the need of screening and early identification of these abnormalities, which if missed can be fatal.

Haematoma complicating subcutaneous fat necrosis of the newborn: a rare complication following therapeutic hypothermia.

A term, large for gestational age male newborn, was admitted to the neonatal intensive care unit with meconium aspiration syndrome and severe hypoxic-ischaemic encephalopathy. The baby was treated with therapeutic hypothermia using a total body cooling blanket. After 48 hours, the baby developed tender, indurated subcutaneous nodules consistent with subcutaneous fat necrosis (SCFN). The lesions started initially over the back but gradually spread to cover both shoulders, upper arms, chest area and both thighs. On day 19 of life, multiple small nodules on the back softened and coalesced to form one sizeable fluctuant swelling over the thoracolumbar area. Over a few hours, the swelling rapidly progressed to a large, tense mass with sloughing of the gangrenous overlying skin. This unusual complication of SCFN required surgical intervention for evacuation and debridement of the haematoma followed by graft repair of the skin defect.

Outcomes of Autologous Fat Grafting in Mastectomy Patients Following Breast Reconstruction.

BACKGROUND: Autologous fat grafting (AFG) is utilized for cosmetic improvement of the reconstructed breast following mastectomy. Fat necrosis (FN), a benign complication of AFG, can raise suspicion of malignancy and require further evaluation. OBJECTIVE: The aim of this study was to determine the incidence of FN in patients who have undergone AFG following mastectomy and reconstruction, and to identify factors contributing to FN. METHODS: A retrospective chart review was conducted of all patients who received AFG following mastectomy and reconstruction at our institution between 2011 and 2016, with a minimum 6-month follow-up period. Patient information, operative details, receipt of radiation, complications, and incidence of cancer recurrence were collected. RESULTS: A total of 171 patients were included in this study. AFG was performed by seven surgeons. Patients received an average of 1.18 treatments, with average follow-up of 26 months. Eighteen patients (10.5%) developed FN an average of 3.4 months following AFG. Patients with a larger volume injected at initial session (p = 0.044) and longer length of follow-up (p = 0.026) had significant increases in risk of developing FN. Core needle biopsy was performed in seven patients and two patients required excision. The rate of cancer recurrence was 1.7% for all patients and 0% in the AFG cohort. CONCLUSIONS: Increased risk of FN following AFG is associated with greater volume injected at the initial session and higher incidence over time. Although AFG is oncologically safe, patients should be counseled on the 10.5% incidence of FN presenting as a palpable abnormality, and the approximately 5% chance of requiring biopsy or excision.

Fat necrosis and polymicrobial wound infection caused partly by Raoultella ornithinolytica after reduction mammoplasty.

Raoultella ornithinolytica is a rare opportunistic aerobic gram-negative bacillus that naturally exists in soil, water and plants. The pathogen has been described in association with diabetic foot infections, biliary infections, bacteraemia and native and prosthetic joint infections. Fat necrosis and wound infection following breast reduction surgery or other plastic surgeries caused by this pathogen have not been previously described. We present a case of bilateral fat necrosis, wound infection and dehiscence in a 24-year-old woman with no significant past medical problems. She initially had an uneventful early postoperative course but 3 weeks after surgery noticed pain and discharge from both nipple/areola area of both breasts which later developed into full-thickness fat necrosis and complete destruction of the nipple areolar complex. R. ornithinolytica, Escherichia coli and Enterococcus faecalis were identified from wound exudate cultures. She was treated with surgical debridement and 2 weeks of appropriate antibiotics with a favourable outcome.

Idiopathic scrotal fat necrosis: an unusual cause of scrotal pain.

The clinical differential diagnosis of scrotal pain with a mass in a prepubertal boy is difficult. Often conditions such as testicular torsion and epididymo-orchitis are diagnosed. We present a rare cause of scrotal pain, idiopathic scrotal fat necrosis. This condition has a typical clinical presentation and ultrasound findings that have been described in a limited number of case reports. However, if it is diagnosed correctly and confirmed with ultrasound, we hope that people will be treated without surgical intervention. We present a case of scrotal wall necrosis and a literature review.

Necrosis grasa subcutánea del recién nacido: reporte de caso y revisión de la literatura / Subcutaneous fat necrosis of the newborn: case report and review of the literature

Resumen: La necrosis grasa subcutánea es una paniculitis poco frecuente, autorresolutiva, que afecta generalmente neonatos de término en las primeras semanas de vida. Se han identificado ciertos factores de riesgo para su desarrollo como asfixia perinatal, hipotermia y aspiración de meconio. Sus complicaciones son infrecuentes, dentro de éstas se destaca la hipercalcemia por su gravedad. Se presenta el caso de un recién nacido que presentó necrosis grasa subcutánea. El diagnóstico se realizó en base a la clínica y el estudio histopatológico. El paciente evolucionó favorablemente con regresión de las lesiones sin complicaciones.

Summary: Subcutaneous fat necrosis is a rare, self-limiting panniculitis, which usually affects full term infants in the first weeks of life. There are several risk factors for its development such as perinatal asphyxia, hypothermia and meconium aspiration. Its complications are unusual, hypercalcemia being the most severe one among all. The study reports the case of a full term new born that presented subcutaneous fat necrosis. The diagnosis was based on clinical findings and histopathological study. The patient improved with regression of lesions, without complications.

Mediastinal (Epipericardial) Fat Necrosis: An Overlooked and Little Known Cause of Acute Chest Pain Mimicking Acute Coronary Syndrome.

Necrosis of the fat adjacent to the heart, referred to as pericardial fat necrosis, epipericardial fat necrosis, and mediastinal fat necrosis, is a rare, self-limited condition. It presents as a sudden onset of severe chest pain that mimics symptoms of pulmonary embolism and acute coronary syndrome. Computed tomography (CT) findings are quite typical and consist of a round- or oval-shaped mass-like lesion containing soft tissue and fat density components in the cardiophrenic space. Lack of familiarity with this condition has led in the past to surgical interventions to remove the mass-like mediastinal fat necrosis. Until the early 2000s, surgical removal of these lesions was deemed the treatment of choice, to exclude a neoplasm. However, the observation that the CT findings of the paracardiac mass resemble fat necrosis seen in other parts of the body and its involution on follow-up CT has led to the realization that the process is a benign and self-limiting one. A dramatic shift in the paradigm of treatment has ensued and surgical treatment is no longer advocated. Awareness of this condition is, therefore, vital so as to diagnose it accurately and avoid unnecessary future surgical interventions.

Keystone-designed buried de-epithelialized flap: A novel technique for obliterating small to moderately sized dead spaces.

Effective obliteration of dead space after reconstructive surgery facilitates a good cosmetic outcome and prevention of delayed wound healing and recurrent infection.We evaluated the efficacy of a keystone-designed buried de-epithelialized (KBD) flap for the obliteration of small to moderately sized surgical dead spaces.We reviewed the medical records of patients who received a KBD flap following removal of a mass or debridement of necrotic tissue from September 2015 to February 2016. The diagnosis, site, dead space dimensions, flap width, drain data, complications, and follow-up duration were recorded.Twenty-eight KBD flaps were evaluated, including 9 cases of fat necrosis, 7 cases of epidermal cyst, and 12 cases of lipoma. Dead space dimensions ranged from 2 × 1.5 × 1 cm to 10 × 5 × 3 cm, with a mean depth of 2.01 cm. Flap sizes ranged from 2.5 × 1 cm to 11 × 3 cm, with a mean flap width of 2.01 cm. No postoperative complications, such as seroma or hematoma, occurred. The cosmetic results were favorable, and all patients were satisfied with their final outcomes.The KBD flap is useful for the obliteration of small to moderately sized surgical dead spaces both spatially and physiologically and shows excellent cosmetic outcomes.

Subcutaneous Fat Necrosis of the Newborn: Report of Five Cases.

Subcutaneous fat necrosis of the newborn (SCFN) is a rare, self-limited disorder of the panniculus which appears in the first few weeks of life. SCFN generally follows an uncomplicated course. However, there are important complications for which the patient must be regularly monitored, including thrombocytopenia, hypoglycemia, hypertriglyceridemia, and most importantly, hypercalcemia. We report five infants with SCFN. All children were born at term. The onset of lesions was between 1 day and 20 days after birth. The back was the most frequent location. Birth hypoxia was the most frequent risk factor. Complications included hypercalcemia, hypoglycemia, and metastatic calcifications (disseminated subcutaneous calcifications, nephrocalcinosis, and myocardial calcifications). This study provides the first case of SCFN in the context of hypoxic encephalopathy and refractory septic shock that required extracorporeal membrane oxygenation (ECMO).

Neutrophil-rich subcutaneous fat necrosis of the newborn: A potential mimic of infection.

BACKGROUND: The inflammatory infiltrate seen in biopsy specimens obtained from patients with subcutaneous fat necrosis of the newborn (SCFN) has classically been described as consisting mostly of histiocytes. However, we encountered patients with SCFN whose biopsy specimens revealed mostly neutrophils, prompting infection to be an initial consideration. OBJECTIVES: We sought to describe cases of SCFN in which neutrophils formed the majority of the infiltrate at our institution and in the literature. METHODS: We performed a retrospective analysis of patients with SCFN reported at our institution and a literature review of SCFN. RESULTS: Thirteen cases of SCFN were identified at our institution. In 2 of 13 cases, neutrophils composed >75% of the inflammatory infiltrate, and both lesions were 1 day old. From the literature review, neutrophils were mentioned as a component of the infiltrate in 10 of 124 cases, but in none were neutrophils described as forming the majority of the infiltrate. LIMITATIONS: This study is limited by its retrospective nature and small sample size. CONCLUSIONS: Neutrophils can comprise most of the inflammatory cells in patients with SCFN, especially early in the course of the disease. This variant of SCFN can be easily mistaken for infection.

Diffuse Dermal Angiomatosis of the Breast With an Apparent Etiology of Underlying Calcified Thrombosed Artery With Adjacent Fat Necrosis.

In this report, the authors present a case of diffuse dermal angiomatosis (DDA) with an underlying mass lesion of the breast, which proved to be a large calcified, thrombosed artery with adjacent fat necrosis. Histologically, DDA consists of hyperplastic vessels, which diffusely infiltrate the papillary and reticular dermis forming small vascular lumina. The condition is associated with various underlying conditions, many of which result in local tissue ischemia. In the past, DDA was most commonly reported on the lower extremities; however, it seems that this entity is more common on the breast than previously recognized. Various treatments have proven beneficial, including revascularization, oral corticosteroids, smoking cessation, and isotretinoin. In this case, our patient benefited from primary excision of the affected area.