Delayed-Onset Sclerema Neonatorum in a Critically Ill Premature Infant.

Sclerema neonatorum is extremely rare in the 21st century. We report a premature infant managed in a neonatal intensive care unit with delayed development of sclerema neonatorum.

Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments.

Sclerema neonatorum (SN) is a rare neonatal panniculitis that typically develops in severely ill, preterm newborns within the first week of life and often is fatal. It usually occurs in preterm newborns with delivery complications such as respiratory distress or maternal complications such as eclampsia. Few clinical trials have been performed to address potential treatments. Successful treatment has been achieved via exchange transfusion (ET), but its use in neonates is declining. Similar to ET, intravenous immunoglobulin (IVIG) enhances both humoral and cellular immunity and thus may decrease mortality associated with SN. We report a case of SN in a term newborn who subsequently developed septicemia. Biopsy showed subcutaneous, needle-shaped clefts without associated necrosis, inflammation, or calcifications. Treatment with IVIG led to notable but short-term clinical improvement. Sclerema neonatorum remains a poorly understood and difficult to treat neonatal disorder. Although IVIG did not prevent our patient's death, further studies are needed to determine its clinical utility in the treatment of this rare disorder.

Panniculitis in children.

This paper will give a comprehensive view of the most frequent panniculitides seen in childhood, with emphasis on the types exclusively found in infancy, and for all other types of panniculitides also found in adults. Aim of this paper is also to analyze the clinical differences between panniculitis in childhood and in adulthood, and to give reliable histopathologic criteria for a specific diagnosis. A review of the literature is here integrated by authors' personal contribution. Panniculitides in children is a heterogeneous group of diseases, as well as in adult life, characterized by inflammation of the subcutaneous fat. Only very few types of panniculitis are exclusively found in childhood, such as Sclerema neonatorum and subcutaneous fat necrosis of the newborn, while the vast majority of the other types may be found both in paediatric age and in adults. Furthermore, this paper will consider in detail panniculitis according to their frequency, such as Erythema nodosum, Lupus panniculitis, Cold panniculitis, panniculitis in Behçet disease, and poststeroid panniculitis. It will also describe rare forms of panniculitis, such as Eosinophilic panniculitis (a pathological entity debated by many authors), Subcutaneous panniculitis T-cell lymphoma, and the different forms of the so call "Lipophagic panniculitis", encompassing respectively the febrile relapsing panniculitis of Weber-Christian disease and the non-relapsing form of Rothmann-Makai disease. For each type of panniculitis considered concise information will be given about epidemiology, etiology, clinical findings, laboratory data, prognosis and therapy, while histopathologic findings will be described in detail.

Recurrent sclerema in a young infant presenting with severe sepsis and severe pneumonia: an uncommon but extremely life-threatening condition.

A one month and twenty-five days old baby girl with problems of acute watery diarrhoea, severe dehydration, severe malnutrition, and reduced activity was admitted to the gastrointestinal unit of Dhaka Hospital of icddr,b. The differentials included dehydration, dyselectrolytaemia and severe sepsis. She was treated following the protocolized management guidelines of the hospital. However, within the next 24 hours, the patient deteriorated with additional problems of severe sepsis, severe pneumonia, hypoxaemia, ileus, and sclerema. She was transferred to the Intensive Care Unit (ICU). In the ICU, she was managed with oxygen supplementation, intravenous antibiotics, intravenous fluid, including a number of blood transfusions, vitamins, minerals, and diet. One month prior to this admission, she had been admitted to the ICU also with sclerema, septic shock, and urinary tract infection due to Escherichia coli and was discharged after full recovery. On both the occasions, she required repeated blood transfusions and aggressive antibiotic therapy in addition to appropriate fluid therapy and oxygen supplementation. She fully recovered from severe sepsis, severe malnutrition, ileus, sclerema, and pneumonia, both clinically and radiologically and was discharged two weeks after admission. Consecutive episodes of sclerema, resulting in two successive hospitalizations in a severely-malnourished young septic infant, have never been reported. However, this was managed successfully with blood transfusion, broad-spectrum antibiotics, and correction of electrolyte imbalance.

A firm plaque on the back of a newborn.

We report a case of subcutaneous fat necrosis of the newborn (SCFN), a rare disorder in term or post-term neonates. Although it is often associated with hematological abnormalities such as anemia and hypercalcemia, SCFN in this patient presented with hyperbilirubinemia. The course of SCFN is generally benign and self-limiting, though may be associated with complications secondary to hypercalcemia.

Risk factors for sclerema neonatorum in preterm neonates in Bangladesh.

BACKGROUND: This study presents a retrospective analysis of risk factors for sclerema neonatorum in preterm neonates in Bangladesh. METHODS: Preterm neonates admitted to Dhaka Shishu Hospital in Bangladesh were enrolled in a clinical trial to evaluate the effects of topical treatment with skin barrier-enhancing emollients on prevention of sepsis and mortality. Four hundred ninety-seven neonates were enrolled in the study and 51 (10.3%) developed sclerema neonatorum. We explored risk factors for sclerema neonatorum by comparing patients with and without sclerema neonatorum. Diagnosis of sclerema neonatorum was based on the presence of uniform hardening of skin and subcutaneous tissues to the extent that the skin could not be pitted nor picked up and pinched into a fold. Cultures of blood and cerebrospinal fluid were obtained in all neonates with clinical suspicion of sepsis. RESULTS: In multivariate analysis, lower maternal education (OR: 1.94; 95% CI: 1.02-3.69; P = 0.043), and signs of jaundice (OR: 2.82; 95% CI: 1.19-6.69; P = 0.018) and poor feeding (OR: 4.71; 95% CI: 1.02-21.74; P = 0.047) on admission were risk factors for developing sclerema neonatorum. The incidence rate ratio of sepsis in neonates who developed sclerema neonatorum was 1.81 (95% CI: 1.16-2.73; P = 0.004), primarily due to Gram-negative pathogens, and risk of death in infants with sclerema neonatorum was 46.5-fold higher (P < 0.001, 95% CI: 6.37-339.81) than for those without sclerema neonatorum. CONCLUSIONS: Sclerema neonatorum was a relatively common, grave condition in this setting, heralded by poor feeding, jaundice, and bacteremia, and signaling the need for prompt antibiotic treatment.

Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka.

BACKGROUND: Sclerema is an uncommon, life-threatening condition, usually of newborns, with a case-fatality rate ranging from 50 to 100%. Very little is known about factors influencing outcome. AIM: To identify clinical and biochemical predictors associated with fatal outcome of sclerema in infants with diarrhoea. METHODS: Thirty infants with sclerema admitted to the Special Care Unit of the Dhaka Hospital of ICDDR,B with diarrhoea from May 2005 to end April 2006 were studied prospectively. Nine infants who died (30%) were considered to be cases while the 21 who survived constituted the comparison group. Hypothermia, severe malnutrition, septic shock, serum ammonia and CRP levels were considered to be predictors of death. Differences in proportions were compared by the chi(2) test and mean differences were compared using Student's t-test or the Mann-Whitney test, as appropriate. RESULTS: The mean age of the 30 infants was 2.1 months (range 12 d to 8 m). Fatal cases were more likely than survivors to be associated with severe underweight, a positive blood culture and higher serum ammonia and serum CRP levels. After adjusting for possible confounders in logistic regression analysis, the likelihood of death was higher in infants admitted with septic shock or who developed it soon after admission (OR 17.96, 95% CI 1.5-0220.4, p=0.024). CONCLUSIONS: Sclerema is associated with a high fatality rate and scleremic infants with diarrhoea who present with septic shock are at a greater risk of death.

Factors associated with sclerema in infants with diarrhoeal disease: a matched case-control study.

AIM: To identify clinical and biochemical factors associated with sclerema in infants with diarrhoeal illness, and their outcome. METHODS: In this case-control study, we enrolled 30 infants with clinical sepsis with sclerema (cases) and another 60, age- and sex-matched infants with clinical sepsis but without sclerema (controls) from among those admitted to the special care unit (SCU) and longer stay unit (LSU) of the Dhaka Hospital of International Centre for Diarrhoeal Disease Research, Bangladesh (ICDDR,B) for their diarrhoeal illness from May 2005 through April 2006. Sclerema as the dependant variable while hypoxia, hypothermia, C-reactive protein (CRP) level, serum total protein and prealbumin level were the major independent variables compared in the analysis. Differences in proportions were compared by the chi-square test and differences of mean were compared by Student's t-test or Mann-Whitney test, as appropriate. RESULTS: The case-fatality was significantly higher among the cases than the controls (30% vs. 2%, CI 2.9-565.5). After adjusting for confounders, infants with sclerema were more likely to be hypothermic (OR 11.6, 95% CI 1.1-126.5), and have lower serum total protein (OR 1.12, 95% CI 1.04-1.21) and prealbumin (OR 1.5, 95% CI 1.1-2.3). CONCLUSION: Diarrhoeal infants having clinical sepsis presenting with hypothermia, lower serum protein and prealbumin are prone to be associated with sclerema.

Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management.

OBJECTIVE: To review published literature on sclerema neonatorum (SN) in order to clarify its clinical presentation, histological features and management compared with two other diseases: subcutaneous fat necrosis of the newborn (SCFN) and scleredema. STUDY DESIGN: PubMed database was searched using the key words Sclerema neonatorum. A total of 55 articles from peer-reviewed journals were reviewed and summarized. RESULT: SN, SCFN and scleredema are diseases of the subcutaneous adipose tissue. SN is characterized by hardening of the skin that gets bound down to the underlying muscle and bone, hindering respiration and feeding and is associated with congenital anomalies, cyanosis, respiratory illnesses and sepsis. Histology of the skin biopsy shows thickening of the trabeculae supporting the subcutaneous adipose tissue and a sparse inflammatory infiltrate of lymphocytes, histiocytes and multinucleate giant cells. SCFN has circumscribed hardening of skin on bony prominences with necrosis of adipocytes in subcutaneous tissue and a dense granulomatous infiltrate on histology. Scleredema is characterized by hardening of the skin along with edema; histology shows inflammatory infiltrate and edema in skin and subcutaneous tissues. SN has a high case fatality rate whereas SCFN and scleredema are self-limiting and lesions resolve within a few weeks to months. Exchange transfusion may improve survival in SN. CONCLUSION: The histological features of skin biopsy should be used to establish diagnosis of SN, SCFN and scleredema as disease-specific treatment is imperative in SN due to high fatality.

Sclerema neonatorum after therapeutic whole-body hypothermia.

Hypothermia is being studied as a neuroprotective therapy after asphyxia. This report is about a term newborn with severe asphyxia who underwent systemic hypothermia (34.5 degrees C) for 72 h. He survived without apparent brain damage but developed sclerema on his back, in the area in contact with the cooling mattress. The sclerema resolved without scarring after three months.

[Adiponecrosis subcutanea neonatorum in congenital pneumonia]. / Adiponecrosis subcutanea neonatorum bei konnataler Pneumonie.

In the course of a connatal pneumonia, a 7-day-old female newborn developed symmetrical subcutaneous nodules on her back, shoulders, and upper arms. These skin lesions were accompanied by hypercalcemia. Histological examination confirmed the putative clinical diagnosis of subcutaneous fat necrosis of the newborn. We discuss the differential diagnoses, therapeutic strategies, and prognosis of this uncommon disorder of the fat tissue.

[Dermatological particularities and pathologies of premature infants]. / Particularités et pathologies dermatologiques du nourrisson prématuré.

In contrast with the full-term infant, the skin of the preterm neonate is structurally and functionally immature, especially birth occurred before 30 weeks gestation. The inefficiency of the epidermal barrier may result in dehydration, thermal instability and toxic reactions from percutaneous absorption of topically applied agents. An increased risk for bacteremia and sepsis exist because of the easily injured skin, combined with compromised immunity. The present article summarizes the consequences of this skin immaturity and the different means to avoid them. We shall also describe 2 pathologies more frequent in premature infants: sclerema neonatorum and acquired zinc deficiency.

Exchange transfusion in septic neonates with sclerema: effect on immunoglobulin and complement levels.

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.

[Observation on the changes of lipid peroxidation in neonates with sclerema].

Thirty-six hospitalized newborn infants with sclerema (case group), which were divided into mild group(n = 18) and severe group(n = 18), and 28 normal neonates (normal control group) were selected to measure the changes of blood lipid peroxidation (LPO) and superoxide dismutase(SOD) so as to study the role of free radicals and lipid peroxide injury the pathogenesis of the entity. The results showed that the plasma LPO contents were significantly increased and SOD were significantly decreased in the case group when compared with those in the normal group (P < 0.01). The plasma LPO was significantly higher and SOD activity was significantly lower in the severe group than those in the mild group (P < 0.01). The study indicates that the free radicals produced in sclerema neonatorum are great in amount and suggests that free radicals possibly participate in the pathogenesis of the disease.