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.

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.

[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.

Sclerema neonatorum and subcutaneous fat necrosis of the newborn.

Sclerema neonatorum and subcutaneous fat necrosis are rare disorders affecting the panniculus of the newborn. This review attempts to put into perspective their similarities and differences in light of historical, biochemical, pathologic, and etiologic considerations. Recent therapeutic modalities and the prognosis are discussed.

Scleredema in an infant.

A 3-month-old infant with scleredema had associated cytomegalovirus pneumonia with a rapidly fatal course. Because differentiation of scleredema from scleredema neonatorum, subcutaneous fat necrosis, and scleroderma may be difficult, histologic diagnosis is important. Cetylpyridinium chloride was used successfully as a fixative to demonstrate acid mucopolysaccharides histologically.

Blood transfusion therapy in the newborn.

This review deals with the various indications, the choice of blood products and the main aspects of their administration for transfusing neonates. Some special problems peculiar to neonatal age, that both neonatologists and blood transfusion services have to take into account, are emphasized. Exchange transfusion in the procedure most frequently used in blood transfusion therapy of neonatal hyperbilirubinaemia of various aetiology, severe anaemia and hyperviscosity due to polycythaemia. The procedure also represents a rational therapeutic approach in the bleeding thrombocytopenic newborn. More recently exchange transfusion has been utilized in the management of DIC, RDS and sepsis. Besides its advantages, metabolic, haemorrhagic and cardiac hazards of this "massive transfusion" are considered. Just as at any other age, the red cell preparation is the blood component most frequently utilized in the transfusion therapy of the neonate, considering not only the treatment of anaemia without hypovolaemic shock, but also the cases of iatrogenic blood loss, a common problem in the high risk neonatal intensive care unit. As transfusion of small increments of blood may often be required for the sick neonate and premature infant, different methods to cope with such conditions are discussed.

Role of exchange transfusion in the treatment of severe septicemia.

Ten critically ill newborn infants presenting with documented septicemia were treated with antibiotics and supportive measures that included assisted ventilation, large blood transfusions and other volume expanders, sodium bicarbonate, and vasoactive drugs. Upon failure of the above treatment to improve the infants' rapidly deteriorating condition and the development of sclerema, exchange transfusions with fresh whole blood were performed and repeated up to four times. Seven of the ten infants showed immediate improvement and ultimately survived. IgM and IgA rose consistently with exchange transfusions. We postulate that these infants improved following exchange transfusion as the result of the removal of endotoxins, improvement of perfusion and of tissue oxygenation, decrease of hemorrhagic complications, and enhancement of the humoral and cellular inflammatory response. The development of sclerema in septicemic newborn infants continues to be an ominous sign despite the use of antibiotics and supportive measures. Our data suggest that exchange transfusions decrease the mortality of this group of critically ill infants.

C3, factor B, alpha-1-antitrypsin in neonatal septicaemia with sclerema.

C3, factor B, and alpha-1-antitrypsin were determined in newborn infants with septicaemia and sclerema, associated with suspected infections, ABO or Rh incompatibility, and hyperbilirubinaemia of unknown origin, during and after treatment with exchange transfusion. Activation products from C3 and factor B, the clearance of the transfused C3, and its synthesis by the recipient were determined also. Infected newborn infants had low levels of C3 and factor B, but a normal amount of alpha-1-antitrypsin. Exchange transfusion lowered the level of alpha-1-antitrypsin and briefly corrected the low level of C3 and factor B. Activation products were formed only exceptionally. As synthesis of C3 is very active, a defective activation of complement pathway linked to an abnormal distribution in extravascular pool is postulated.