ABSTRACT
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.
Subject(s)
Humans , Infant , Male , Follow-Up Studies , Genitalia , Histiocytes , Lymphocytes , Nipples , Prognosis , Rare Diseases , Sclerema Neonatorum , Skin , Subcutaneous FatSubject(s)
Humans , Female , Infant, Newborn , Neoplasms, Adipose Tissue , Adipose Tissue/abnormalities , Sclerema Neonatorum , PanniculitisABSTRACT
A prospective study was carried out to determine the predictors of outcome in neonates with sepsis admitted to neonatal care unit at Basrah Maternity and Children Hospital over six months [from the first of November 2004 till the end of April 2005]. One-hundred twenty neonates were studied, sepsis was confirmed by clinical and laboratory measures. Seventy four [61.7%] neonates were males and 46 [38.3%] were females. Thirty three [27.5%] were preterm and 87 [72.5%] were full term. Sixty seven [55.8%] neonates were still alive during period of hospitalization and discharged home, while fifty three [44.2%] neonates died. Early onset sepsis was detected in 35[29.2%] neonates while late onset sepsis was detected in 85[70.8%] neonates, however, the mortality rate was higher in early onset sepsis [62.9%] compared to late onset sepsis [36.5%]. The mean body weight was significantly lower in neonates who died [1.97 +/- 0.67], compared to those who survived [2.79 +/- 0.6]. A significantly higher mortality rates were among premature neonates [69.7%], and those with intrauterine growth retardation [70.8%]. In addition, the death rate was higher in neonates with maternal history of prolonged rupture of membrane >/= 24 hours [61.5%] compared to [39.4%] in neonates with maternal history of rupture membrane of < 24 hours before labor. The clinical signs that predict high mortality were sclermic skin [94.2%], signs of dehydration [82.8%] and prolonged capillary refilling time [68%]. Highest mortality was associated with positive blood culture for Pseudomonas aeruginosa and Staphylococcus aureus, where all neonates died [100%], followed by klebsiella spp. and Escherichia coli [71.1%] and [48.5%] respectively in comparison with neonates who have positive blood culture for Proteus and Enterobacter aeruginosa where only 7.9% and 11.1% of neonates died respectively. A statistically significant higher mortality was reported in neonates having thrombocytopenia, neutropenia and C-reactive protein >/-10 mg / dl. Regression analysis of different neonatal and maternal variables, hematological and microbiological tests, revealed that body weight, gestational age, thrombocytopenia, neutropenia, positive blood culture for klebsiella spp., prolonged capillary refilling time, sclerma and signs of dehydration are predictive factors of the outcome of death in neonatal sepsis
Subject(s)
Humans , Male , Female , Sepsis/microbiology , Sepsis/blood , Prognosis , Prospective Studies , Intensive Care Units, Neonatal , Time Factors , Survival Analysis , Birth Weight , Infant Mortality , Sclerema Neonatorum , Infant, Newborn , Risk Assessment , Pseudomonas , ThrombocytopeniaABSTRACT
Subcutaneous fat necrosis of newborn is an uncommon disorder characterized by firm subcutaneous plaques and nodules usually appearing shortly after birth. A female newborn with brain death developed diffuse hardening of trunk, extremity, cheek and buttock in the first 5 weeks of the life. A clinical diagnosis of sclerema neonatorum was made. But histologic examination revealed patchy areas of fat necrosis with crystallization, infiltration of histologic cells and several multinucleated giant cells containing needle-shaped clefts. Calcium deposits were also demonstrated within the necrotic area. So, we diagnosed this case as subcutaneous fat necrosis of newborn with generalized hardening of the skin.
Subject(s)
Female , Humans , Infant, Newborn , Brain Death , Buttocks , Calcium , Cheek , Crystallization , Diagnosis , Extremities , Fat Necrosis , Giant Cells , Necrosis , Parturition , Sclerema Neonatorum , Skin , Subcutaneous FatABSTRACT
Las lesiones cutáneas en recién nacidos son frecuentes, algunas de ellas no tienen mayor importancia clínica o son transitorias, mientras que otras pueden constituir un marcador cutáneo de otra enfermedad o requerir un control periódico por sus características evolutivas. Con frecuencia estas lesiones son subvaloradas o subdiagnosticadas, por lo que se hace importante advertir al personal de salud la trascendencia, el pronóstico o el riesgo eventual que pueden representar para el paciente la aparición de dichas lesiones
Subject(s)
Humans , Infant, Newborn , Infant, Newborn, Diseases/classification , Skin Diseases/classification , Acne Vulgaris/diagnosis , Diagnosis, Differential , Ectodermal Dysplasia/diagnosis , Fat Necrosis/diagnosis , Sebaceous Glands/pathology , Hyperplasia/diagnosis , Jaundice/diagnosis , Lupus Erythematosus, Cutaneous/diagnosis , Melanosis/diagnosis , Miliaria/classification , Miliaria/diagnosis , Sclerema Neonatorum/diagnosisABSTRACT
OBJECTIVE: To study the effect of exchange transfusion (ET) on the levels of immunoglobulins (Ig) and C3 in neonatal sepsis with sclerema. DESIGN: Randomized controlled trial in a referral neonatal unit of a teaching hospital. SUBJECTS: Consecutive culture positive septic neonates with sclerema were enrolled and were randomized to undergo ET (study group, n = 20) or no ET (controls, n = 20). RESULTS: Mortality was 50% in the study group and 95% in controls. Gram negative organisms accounted for 85% in study group and 90% in controls. IgG, IgA and IgM levels rose significantly while C3 levels did not show significant rise 12-24 hours after ET. Ig and C3 levels did not change significantly in the controls. CONCLUSION: ET with fresh whole blood in septicemic newborns with sclerema improves survival, particularly in the more premature group and significantly enhances, IgG, IgA and IgM levels.
Subject(s)
Bacteremia/complications , Complement System Proteins/metabolism , Exchange Transfusion, Whole Blood , Female , Humans , Immunoglobulins/blood , Infant, Newborn , Infant, Premature , Male , Sclerema Neonatorum/complications , Statistics, Nonparametric , Survival RateABSTRACT
Histopathological study of 10 cases sclerema neonatorum showed subcutaneous fibrosis with lobulation in all the cases. 8 (80 percent) cases showed non-specific chronic inflammation of dermis and thinning of epidermis with atrophy of rete pegs and hypercollgenisation of dermis was evident in 70 percent cases. Fat necrosis with crystallisation of fat content was not a conspicuous feature.
Subject(s)
Adipose Tissue/pathology , Cell Size , Epidermis/pathology , Fat Necrosis/pathology , Fetal Death/pathology , Humans , Infant, Newborn , Inflammation , Sclerema Neonatorum/pathologyABSTRACT
Os autores apresentam dois casos de adiponecrose do recem-nascido em duas criancas: uma menina de 2 meses e um menino de 32 dias.Trata-se de patologia caracterizada por areas endurecidas no tecido subcutaneo com formacao de nodulos irregulares e de consistencia dura, localizando-se em proeminencias osseas da face, ombros, membros superiores, nadegas e membros inferiores. Histopatolicamente ocorreu necrose subcutanea gordurosa, com celulas gigantes de tipo corpo estranho e deposito de cristal. Foi feito um diagnostico diferencial com a paniculite ao frio e esclerema neonatorum. A importancia da apresentacao baseia-se na raridade; na evolucao benigna sem tratamento especifico; no fato de ter sido evitada terapeutica intempestiva (corticoterapia) devido ao pouco conhecimento sobre a entidade
Subject(s)
Sclerema Neonatorum , Fat NecrosisABSTRACT
We were experienced a case of sclerema neonatorum which was developed in a 13 days old newborn, and we report it with a brief review.
Subject(s)
Humans , Infant, Newborn , Sclerema NeonatorumABSTRACT
Sclerema neonatorum is an uncommon and fatal disease which encountered in premature & debilitated newborn infants. Recently we experienced a case of sclerema neonatorum in premature infant with congenital heart disease which was confirmed by autopsy. We present a case with a brief review of literatures.
Subject(s)
Humans , Infant, Newborn , Autopsy , Heart Defects, Congenital , Infant, Premature , Sclerema NeonatorumABSTRACT
Sclerema neonatorum is a lesion of degeneration, necrosis or crystallization of adipose tissue of new born, and is classified into two types; the generalized type in which death occurs usually within one week without treatment, and the nodular type which is localized and heals spontaneously. In this report, nodular type was presented. Nut to palm sized indurated plaques on deltoid region, upper back and hip were noted 3 weeks after birth. Histopathologic findings of necrosis of subcutaneous tissue with early granulomatous reaction were showed in biopsy specimen. Literature was briefly reviewed for the discussion of clinical and microscopic findings of this disease.