Subcutaneous fat necrosis of the newborn presenting as circular alopecia: a novel presentation.

Subcutaneous fat necrosis (SCFN) is an inflammatory disorder of the adipose tissue that commonly presents in neonates as tender subcutaneous nodules on the trunk and extremities with hypercalcemia as a potential complication. We report the case of a 3-month-old female who presented with circular alopecia of the scalp and was found to have histopathologically confirmed SCFN. Recognition of SCFN as a cause for alopecia in young infants may allow prompt screening for hypercalcemia and reduce the risk for associated morbidity and mortality.

Subcutaneous fat necrosis of the newborn and associated hypercalcemia: A systematic review of the literature.

Subcutaneous fat necrosis of the newborn is an uncommon disorder, and although usually benign, associated hypercalcemia can lead to complications such as failure to thrive and renal failure. Many sources suggest screening for hypercalcemia for 6 months following resolution of skin lesions, but little data are available to support this recommendation. This study examines existing published literature to better guide practitioners regarding screening evaluations of asymptomatic patients with subcutaneous fat necrosis. A systematic review of the literature was conducted using a PubMed English literature search. Data from case reports and case series were collected regarding the presence of hypercalcemia and associated complications, birth history, and age of onset/resolution of skin lesions and laboratory abnormalities. Approximately half (51%) of infants reported had hypercalcemia. Most (77%) developed detectable hypercalcemia within 30 days of skin lesion onset, and 95% developed detectable hypercalcemia within 60 days of skin lesion onset. Hypercalcemia was detected in only 4% of patients > 70 days following onset of skin lesions. Seventy-six percent had resolution of hypercalcemia within 4 weeks of detection. Hypercalcemia was more prevalent in full-term vs pre-term infants (P-value = 0.054), and higher birthweight was significantly associated with an increased risk of developing hypercalcemia (P-value = 0.022). Although gestational age trended toward significance, the only statistically significant clinical feature predicting the development of hypercalcemia was higher birthweight. Current recommendations for laboratory monitoring are not evidence-based, and this study provides interim data to guide practitioners until prospective, randomized controlled trials are conducted.

A Case Study of Severe Hypercalcemia Secondary to Subcutaneous Fat Necrosis: A Diagnosis of Exclusion.

Subcutaneous fat necrosis (SCFN) is a rare complication, usually occurring in otherwise healthy full-term infants who have experienced some level of trauma that causes ischemic injury to adipose tissue. Tissue injury usually occurs in areas of the body that are exposed to excessive pressure as during delivery. Tissue injury has also been described secondary to therapeutic cooling. This case study presents an infant who received whole body cooling for hypoxic ischemic injury and later developed severe hypercalcemia at one month of age without the skin lesions consistent with SCFN. The differential diagnosis for hypercalcemia and how it relates to SCFN is presented, as well as clinical presentation, treatment, and prognosis.

Pancreatic panniculitis: the "bright" side of the moon in solid cancer patients.

BACKGROUND: Pancreatic panniculitis is a rare complication of pancreas disorders occurring in 0.3-3% of patients, most often accompanied by the pancreatic acinar carcinoma. It presents multiple, painful, deep, ill-defined, red-brown, migratory nodules and plaques of hard elastic consistency; often ulcerated and typically located on the lower proximal and distal extremities. The pathogenesis is not fully understood, but it is thought to result from lipolysis and fat necrosis with secondary tissue inflammation induced by pancreatic enzymes. Histopathology shows subcutaneous lobular fat necrosis with anuclear adipocytes (called ghost cells) surrounded by a mixed inflammatory infiltrate. Focal calcification may also be seen. The treatment is directed to the underlying disorder, which may result in regression of skin lesions. CASE PRESENTATION: We present two cases of pancreatic panniculitis with similar clinical, laboratory, and histopathological features associated with different internal malignancy. The first case, after extensive investigations showed the presence of a pancreatic carcinoma with multiple liver metastases and a poor prognosis. The second one instead is the first case in literature where painful subcutaneous nodules of the legs were the early manifestation of a neuroendocrine carcinoma of the adrenal gland. CONCLUSIONS: Although subcutaneous fat necrosis usually occurs late in the course of a malignancy, recognition of the association with pancreatic panniculitis may prevent a long delay in the diagnosis and management of the occult neoplasm. It should be primarily considered when panniculitis is widespread and persistent, and frequent relapses or tendency to ulcerate of the nodules are regarded as red flags.

Panniculitis: A summary.

The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of "biological" therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda. Polyarteritis nodosa and Behçet's disease are the conditions that are based in the subcutaneous septa with vasculitis. Predominantly-lobular panniculitides with no vasculitis include pancreatogenic panniculitis, the panniculitis of alpha-1-antitrypsin deficiency, panniculitis associated with lupus erythematosus and dermatomyositis, subcutaneous Sweet syndrome, eosinophilic panniculitis, factitial panniculitis, cold panniculitis, panniculitis following injections of corticosteroids, lipomembranous (ischemic) panniculitis; sclerema neonatorum and subcutaneous fat necrosis of the newborn, and Rosai-Dorfman disease of the subcutis. Erythema induratum and infectious panniculitis are vasculitic and lobulocentric conditions. This article reviews the histological features of these diseases.

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.

Macrosomia and haemodynamic instability may represent risk factors for subcutaneous fat necrosis in asphyxiated newborns treated with hypothermia.

AIM: To identify additional risk factors other than asphyxia and hypothermia in newborns developing subcutaneous fat necrosis (SCFN). METHODS: We conducted a prospective cohort study of all term asphyxiated newborns treated with hypothermia from 2008 to 2015. The presence and location of SCFN were recorded at the time of discharge or at follow-up visits. To identify the risk factors for developing SCFN, we compared the perinatal characteristics of those newborns who developed SCFN with those who did not. RESULTS: The newborns developing SCFN had significantly higher birthweights compared with those newborns who did not develop SCFN. Among the newborns with a birthweight equal or superior to the 90th percentile, those who developed SCFN had a significantly higher use of inotropic support and higher maximum troponin levels during their initial hospitalisation. CONCLUSION: A higher birthweight represented an independent risk factor for developing SCFN in asphyxiated newborns treated with hypothermia. When macrosomia is present, other risk factors related to haemodynamic instability during the initial hospitalisation may also increase the risk of developing SCFN.

Subcutaneous fat necrosis of the newborn: A systematic review of surgical management and outcomes.

BACKGROUND: Subcutaneous fat necrosis of the newborn (SCFN) is a rare form of panniculitis manifesting as erythematous plaques or nodules at sites of brown fat in neonates. Surgical management may be indicated in severe cases; however, there is a paucity of literature compiling presentations and outcomes of these surgical patients. METHODS: The authors performed a systematic review, in consultation with a licensed librarian, on MEDLINE and Embase for studies including patients with SCFN who were surgically managed. RESULTS: The search strategy generated 705 results, among which 213 (30.2%) were excluded for lack of discussion on surgical management. Twenty-two studies discussed surgical management of SCFN in 26 patients, but in 6 of these studies the patients were not surgically managed. Ultimately, 16 articles with 16 patients who were surgically managed were included in the study. Average age at diagnosis was 11.8 ± 9.8 days; average age at surgery was 39.5 ± 70.4 days. The most common etiologies were "unknown" (6, 37.5%), therapeutic hypothermia (4, 25.0%), and birth complications (4, 25.0%). Patients harbored nodules on the back (14, 87.5%), upper extremities (7, 43.8%), lower extremities (7, 43.8%), buttocks (5, 31.3%), and head or neck (3, 18.8%). Linear regression models revealed the presence of back lesions and predicted concomitant medical complications (ß = 2.71, p = 0.021). CONCLUSIONS: Patients undergoing surgical management for SCFN most commonly harbor lesions on the back and extremities that are secondary to therapeutic hypothermia or of unknown origin. Reporting of additional cases is needed to further elucidate surgical management and outcomes.

A Hypercalcemic Crisis Complicating Subcutaneous Fat Necrosis of the Newborn: A Case Report and Literature Review.

Subcutaneous fat necrosis of the newborn (SFNN) is a rare panniculitis that is characterized by the presence of skin nodules. Although SFNN is a self-limited benign disease, effective follow-up is highly recommended to detect hypercalcemia and other complications early on. A male newborn was admitted twice to the neonatal intensive care unit (NICU). The first NICU admission was for hypoglycemia, and the second was due to late-onset sepsis, in which reddish nodules were detected on the back, flanks, shoulders, and posterior aspects of the legs. At 44 days old, the infant was referred to the emergency department due to a hypercalcemic crisis. Screening for other SFNN complications revealed eosinophilia, hypoglycemia, and nephrocalcinosis. The hospitalization was further complicated by a rare occurrence of deep venous thrombosis. The calcium level was followed up to ensure the patient's recovery. This case highlights the complications that might follow SFNN and emphasizes the importance of its surveillance.

Subcutaneous Fat Necrosis of the Newborn After Whole-Body Cooling for Birth Asphyxia.

Therapeutic hypothermia (TH) is a challenging treatment for a neonate who suffers from hypoxic-ischemic encephalopathy. It has been shown to improve neurodevelopmental outcomes and survival in infants with moderate-to-severe hypoxic-ischemic encephalopathy. However, it has severe adverse effects such as subcutaneous fat necrosis (SCFN). SCFN is a rare disorder that affects term neonates. It is a self-limited disorder but can have severe complications such as hypercalcemia, hypoglycemia, metastatic calcifications, and thrombocytopenia. In this case report, we present a term newborn who developed SCFN after whole-body cooling.

Safety and Efficacy of Pamidronate in Neonatal Hypercalcemia Caused by Subcutaneous Fat Necrosis: A Case Report.

Subcutaneous fat necrosis of the newborn (SCFN) is a panniculitis that develops in fatty areas after fetal or perinatal distress. Prognosis is generally good with complete regression, but it can be complicated by metabolic abnormalities like hypoglycemia, hypertriglyceridemia, thrombocytopenia, and also potentially life-threatening hypercalcemia. Treatments have included hydration, furosemide and corticosteroids. These treatments can be prolonged for several days and can have complications such as nephrocalcinosis. Use of bisphosphonates has been rarely reported in newborn. We describe a case of severe hypercalcemia complicating subcutaneous fat necrosis in a newborn successfully treated by a single dose of pamidronate after having obtained partial response by therapy with hyperhydration, furosemide and hydrocortisone. When high levels of calcium do not respond to first line therapy with hyperhydration and diuretic therapy, bisphosphonates treatment could be considered a valid choice to treat hypercalcemia and to avoid corticosteroids. Further studies are needed to understand if pamidronate and other bisphosphonates can be considered the first choice in hypercalcemia due to SCFN.

The cause of severe hypercalcaemia resistant to pamidronate treatment: subcutaneous fat necrosis with no visible skin lesion.

Subcutaneous fat necrosis (SCFN) is an uncommon cause of neonatal hypercalcaemia. It is usually seen in neonates after a complicated delivery within the first month of life. While uncommon, hypercalcaemia can be fatal. It is characterised by red-purple plaques in fatty points along with firm subcutaneous nodules. Rarely, SCFN may cause severe hypercalcaemia with no visible skin lesion. In this rare case, we report severe infancy hypercalcaemia without characteristic skin lesion on first physical examination, unresponsive to hydration, diuretic, prednisolone and standard dose of pamidronate treatment. As timely diagnosis and treatment are so important, this complication should be kept in mind even in such clinical presentations.

Subcutaneous fat necrosis in newborns: a systematic literature review of case reports and model of pathophysiology.

BACKGROUND: Subcutaneous fat necrosis of the newborn (SCFN) is a rare disease occurring in the first days of life. Characteristically, the infants show hard nodules in subcutaneous tissue, purple or erythematous in color and appear on the upper back, cheeks, buttocks and limbs. In most cases, SCFN is a self-limiting disease, as the nodules disappear in up to 6 months. A severe complication associated with SCFN is hypercalcaemia. Pathophysiological mechanisms causing SCFN or associated hypercalcaemia are not fully understood yet. METHODS: A systematic literature research including the six biggest databases for medical research has been used to identify all published case reports of SCFN. N = 206 publications has been identified containing n = 320 case reports. All cases have been classified into four subgroups (depending on reported serum-calcium-level): hypercalcaemia, normocalcaemia, hypocalcaemia or no information given. Reported maternal factors, birth characteristics, details about SCFN, diagnostics, therapy and long-term observations have been extracted from publications. RESULTS: This is the first systematic literature research that summed up all published cases of SCFN from 1948 up to 2018. Information about serum calcium level was given in 64.3% of the cases. From those, the majority showed hypercalcaemia (70.5%) (normocalcaemia 25.1%, hypocalcemia 4.3%). 89.3% of newborns with hypercalcaemia showed suppressed levels of the parathormone. Maternal gestational diabetes, maternal hypertensive diseases during pregnancy, macrosomia (> 4000g), asphyxia and therapeutic hypothermia are risk factors for SCFN. Histological findings showed a granulomatous inflammation in 98% of cases. CONCLUSION: We identified that maternal, birth characteristics and therapeutic measures are probably risk factors for SCFN. These risk factors should be taken into account within the care of neonates.

Atypical facial presentation of subcutaneous fat necrosis of the newborn.

Subcutaneous fat necrosis of the newborn (SCFN) is a rare self-limiting panniculitis. It is thought to be associated with perinatal hypoxia and therapeutic hypothermia. It is characterised by firm subcutaneous nodules on the back, shoulder and arms. We present a rare facial presentation of SCFN in a 4-week-old infant with no history of therapeutic cooling. She presented with a discrete right cheek mass with no overlying skin changes. We present the diagnostic challenge and undertake a review of the literature. SCFN is an important differential diagnosis in a neonate with subcutaneous facial lesions. SCFN can be complicated by metabolic derangements including hypercalcaemia.

Clinicopathological features of neonatal subcutaneous fat necrosis and its implications.

Not required for Clinical Vignette.

Subcutaneous fat necrosis of the newborn - An atypical case with typical complications.

Subcutaneous fat necrosis of the newborn should be considered in newborns with suggestive skin lesions, even in the absence of perinatal distress. SCFN may cause long-standing complications, like hypertriglyceridemia or hypercalcemia. Hypercalcemia can be refractory to therapy and lead to poor weight gain and nephrocalcinosis, which should be closely monitored.

Subcutaneous fat necrosis associated with hypercalcemia in neonates with neonatal encephalopathy treated with therapeutic hypothermia.

Subcutaneous fat necrosis of the newborn (SFNN) is a rare, self-resolving panniculitis. The onset of skins lesions occurs within the first week of life, with a median age of onset around day 6 of life (range 1-70). About 50% of neonates with SFNN will develop hypercalcemia in the first month though some present later. Typically, SFNN develops prior to hypercalcemia. Only half of the neonates have classic symptoms of hypercalcemia; routine screening for hypercalcemia is recommended for neonates with SFNN or at-risk. The mechanism for hypercalcemia is usually aberrant 1,25-dihydroxyvitamin D synthesis in the necrotic tissue increasing intestinal absorption of calcium. Prompt recognition and treatment is required, often in hospital. Treatment options are low calcium-vitamin D formula or sometimes intravenous bisphosphonates. Regular monitoring post-intervention is required when normalizing the diet. Outcomes are good with routine surveillance and interventions. A multidisciplinary team approach (neonatologists, pediatric endocrinologists and nephrologists, dieticians) is optimal.

Severe hypercalcaemia and acute renal failure in an infant with subcutaneous fat necrosis.

Subcutaneous fat necrosis (SFN) in the newborn is a form of panniculitis which presents with erythematous nodules and indurated plaques. Severe life-threatening hypercalcaemia can occur as a late complication. A 2-month-old girl presented with severe hypercalcaemia and acute renal injury as a complication of SFN. She was admitted to hospital with the chief complaint of failure to thrive. She had a history of therapeutic hypothermia. After successful treatment of the hypercalcaemia with bisphosphonates, the acute renal injury recovered spontaneously. In neonates with SFN, acute renal injury is a rare complication of hypercalcaemia. Timely prevention of the complications of hypercalcaemia in SFN is essential.

What to Look Out for in a Newborn with Multiple Papulonodular Skin Lesions at Birth.

Multiple papulonodular skin lesions at birth can indicate the presence of various benign and malignant disorders. Although the lesions' clinical aspect (color and consistency, in particular) may steer the clinician towards one disorder or another (infantile myofibromatosis, xanthogranuloma, or metastatic neuroblastoma), the diagnosis can only be confirmed by the histopathologic assessment of a biopsy. In neonates, a rapid but accurate diagnosis is critical because skin lesions may be the first manifestation of a malignant disorder like leukemia cutis or metastatic neuroblastoma. Here, we review the various disorders that may manifest themselves as multiple skin lesions at birth.