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Dermatology Online Journal Nov 2020Subcutaneous fat necrosis of the newborn is a rare lobular panniculitis. It is characteristic of term or post-term neonates with a history of perinatal compromise or...
Subcutaneous fat necrosis of the newborn is a rare lobular panniculitis. It is characteristic of term or post-term neonates with a history of perinatal compromise or maternal gestational pathology. The cutaneous manifestations consist of erythematous and indurated subcutaneous plaques and nodules located over the dorsal region and the shoulders. Diagnosis is clinical and pathological. Histopathological findings include lobular panniculitis with a lymphohistiocytic inflammatory infiltrate with few neutrophils, fatty necrosis, deposition of radial needle-shaped crystals in the adipocytes, and possible calcification and hemorrhage. The cutaneous ultrasound shows hyperechoic and avascular subcutaneous cellular tissue and acoustic shadows may appear corresponding to calcifications. The clinical differential diagnosis includes sclerema neonatorum and post-corticosteroid panniculitis. Histologically crystal-forming panniculitis conditions are in the differential diagnosis. The disease is usually self-limited but complications such as hypercalcemia, hypoglycemia, hypertriglyceridemia, thrombocytopenia, and anemia may occur. Complications should be ruled out and treated at diagnosis and during follow-up. The most important complication is hypercalcemia.La necrosis grasa subcutánea neonatal es una paniculitis lobulillar infrecuente. Es característica de neonatos a término o postérmino con antecedentes de sufrimiento perinatal o patología gestacional materna. Las manifestaciones cutáneas consisten en placas y nódulos subcutáneos eritematosos e indurados localizados preferentemente en la región dorsal y los hombros. El diagnóstico es clínicopatológico. Los hallazgos histopatológicos comprenden una paniculitis lobulillar con un infiltrado inflamatorio linfohistiocitario con escasos neutrófilos, necrosis grasa, depósito de cristales radiados en los adipocitos y posibles focos de calcificación y hemorragia. En la ecografía cutánea se observa hiperecogenicidad y avascularización del tejido celular subcutáneo y pueden aparecer sombras acústicas posteriores que se correspondencon calcificaciones. El diagnóstico diferencial clínico se debe realizar con el escleredema neonatorum y la paniculitis postesteroidea, e histológicamente con las paniculitis con formación de cristales. La enfermedad suele ser autolimitada pero pueden aparecer complicaciones como la hipercalcemia, la hipoglucemia, la hipertrigliceridemia, latrombocitopenia y la anemia. Las complicaciones deben ser descartadas y tratadas en el diagnóstico y durante el seguimiento. La complicación másimportante es la hipercalcemia.
Topics: Back; Female; Humans; Infant, Newborn; Neck; Subcutaneous Fat; Ultrasonography
PubMed: 33342185
DOI: No ID Found -
The Journal of Investigative Dermatology May 1965
Topics: Adipose Tissue; Fatty Acids; Glycerides; Histocytochemistry; Histological Techniques; Humans; Infant; Infant, Newborn; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Sclerema Neonatorum
PubMed: 14290303
DOI: No ID Found -
Proceedings of the Royal Society of... Dec 1959
Topics: Humans; Infant, Newborn; Lipodystrophy; Sclerema Neonatorum
PubMed: 13820009
DOI: No ID Found -
Proceedings of the Royal Society of... Jul 1953
Topics: Humans; Infant, Newborn; Medical Records; Sclerema Neonatorum
PubMed: 13074213
DOI: No ID Found -
Annals of Dermatology Dec 2017Sclerema neonatorum (SN) is a rare disease characterized by firm, indurated, waxy skin lesions extending throughout the body, sparing the fat-free soles, palms, and...
Sclerema neonatorum (SN) is a rare disease characterized by firm, indurated, waxy skin lesions extending throughout the body, sparing the fat-free soles, palms, and genitalia. The prognosis of SN is generally very poor, with a high fatality rate. We report the case of a full-term infant with delayed onset of SN showing good prognosis. A 4-month-old Korean male infant presented with diffuse waxy, sclerotic skin lesions on the whole body, sparing the face, nipples, and genital area, which began developing at 2 months of age. Histopathologic findings of the sclerotic skin lesions showed wide, fibrous intersecting bands in the subcutaneous fat tissue. Only sparse infiltration of lymphocytes and histiocytes was observed in the fat lobules and septa. Based on clinical presentation and histopathologic findings, he was diagnosed with SN. The patient survived with conservative care and had mild improvement of the skin lesions on his follow-up visit at 12 months of age.
PubMed: 29200772
DOI: 10.5021/ad.2017.29.6.790 -
Case Reports in Pediatrics 2020Sclerema neonatorum (SN) is a rare form of panniculitides with an obscure incidence, aetiology, and pathogenesis. This condition is peculiar to preterm neonates,...
Sclerema neonatorum (SN) is a rare form of panniculitides with an obscure incidence, aetiology, and pathogenesis. This condition is peculiar to preterm neonates, especially those with comorbidities such as sepsis, congenital anomalies, congenital heart disease, and gastrointestinal and respiratory diseases. Usually, it presents within the first seven days of life, but may develop a few weeks later. Typically, SN manifests with diffuse hardening of the skin and subcutaneous tissues that subsequently bind to the underlying muscle and bone, often beginning in the buttocks, thighs, or trunk, and progressing to other body parts, but sparing the soles, palms, and genitalia. Diagnosis is based on clinical characteristics. It has been associated with a high mortality, despite aggressive use of various treatment modalities such as antibiotics, steroids, fresh blood transfusion, exchange blood transfusion, and intravenous immunoglobins. This is a report of a macrosomic term neonate who presented with typical clinical and histopathological features of SN.
PubMed: 33489400
DOI: 10.1155/2020/8837064 -
Skin Health and Disease Oct 2023Sclerema neonatorum (SN) is a rare condition of neonatal panniculitis with a poor prognosis and a high fatality rate. It clinically presents as hardening of the skin and...
Sclerema neonatorum (SN) is a rare condition of neonatal panniculitis with a poor prognosis and a high fatality rate. It clinically presents as hardening of the skin and subcutaneous adipose tissue extending throughout the body, sparing the fat-free soles, palms, and genitalia. SN typically affects gravely ill, preterm neonates in the first week of life and diagnosis is often clinical. We report a case of an eight-day-old premature infant diagnosed with early-onset neonatal sepsis who presented with clinical and histopathological features of SN. Despite early treatment of the sepsis with intravenous antibiotics and the SN with a topical corticosteroid cream and moisturiser, the infant died on the twelfth day of life.
PubMed: 37799354
DOI: 10.1002/ski2.255 -
Frontiers in Pediatrics 2021Neonatal gastric perforation is a rare but life-threatening issue. The aim of this study was to describe the clinical characteristics and prognosis of patients with...
Neonatal gastric perforation is a rare but life-threatening issue. The aim of this study was to describe the clinical characteristics and prognosis of patients with neonatal gastric perforation and identify predictive factors for poor prognosis. This was a retrospective cohort study of patients with neonatal gastric perforation treated in a tertiary pediatric public hospital between April 2009 and October 2020. The enrolled patients were divided into survival and non-survival groups. Demographic information, clinical characteristics, laboratory and imaging features, and outcomes were collected from the electronic medical record. Univariate and multivariate logistic regression analyses were performed to obtain the independent factors associated with death risk. Additionally, we separated this population into two groups (pre-term and term groups) and explored the mortality predictors of these two groups, respectively. A total of 101 patients with neonatal gastric perforation were included in this study. The overall survival rate was 70.3%. Seventy-one (70.3%) were pre-term neonates, and sixty-two (61.4%) were low-birth-weight neonates. The median age of onset was 3 days (range: 1-11 days). Abdominal distension [98 (97.0%) patients] was the most common symptom, followed by lethargy [78 (77.2%) patients], shortness of breath [60 (59.4%) patients] and vomiting [34 (33.7%) patients]. Three independent mortality risk factors were identified: shock (OR, 3.749; 95% CI, 1.247-11.269; = 0.019), serum lactic acid > 2.5 mmol/L (5.346; 1.727-16.547; = 0.004) and platelet count <150 × 10/L (3.510; 1.115-11.053; = 0.032). There was a borderline significant association between sclerema neonatorum and total mortality (4.827; 0.889-26.220; = 0.068). In pre-term infants, serum lactic acid > 2.5 mmol/L and platelet count <150 × 10/L remained independent risk factors for death. In term infants, the incidence of shock, coagulopathy, pH < 7.3, serum lactic acid > 2.5 mmol/L, and hyponatremia were statistically different between non-survival and survival groups. Shock, hyperlactatemia, and thrombocytopenia are independently associated with an increased risk of death in patients with neonatal gastric perforation. Identification of modifiable risk factors during the critical periods of life will contribute to the development of effective prevention and intervention strategies of neonatal gastric perforation.
PubMed: 34055689
DOI: 10.3389/fped.2021.652139 -
Journal of Clinical Pathology Mar 1966The fatty crystals in three previously published cases of sclerema neonatorum were identified by means of x-ray diffraction as sodium stearate and palmitic acid. A...
The fatty crystals in three previously published cases of sclerema neonatorum were identified by means of x-ray diffraction as sodium stearate and palmitic acid. A composite view of the pathogenesis of both sclerema neonatorum and adiponecrosis cutis neonatorum is discussed.
Topics: Adipose Tissue; Humans; In Vitro Techniques; Infant; Infant, Newborn; Necrosis; Palmitic Acids; Sclerema Neonatorum; Stearic Acids; X-Ray Diffraction
PubMed: 5909702
DOI: 10.1136/jcp.19.2.193 -
Proceedings of the Royal Society of... Apr 1931
PubMed: 19988035
DOI: No ID Found