1/10. Exchange transfusion in severe neonatal infection with sclerema. Two critically ill newborn babies with severe infection associated with sclerema were successfully treated with appropriate antibiotics and repeated exchange transfusions. ( info) |
| sclerema neonatorum (SN) is a rare disease of the newborn characterised by diffuse hardening of the subcutaneous adipose tissue. The condition is distinct from the localised violacious nodules observed in subcutaneous fat necrosis of the newborn (SCFN). Both conditions were diagnosed within the first 2 weeks of life in the same infant. SN and SCFN are unusual conditions in the newborn, the former usually associated with a severe clinical illness, following delivery or major surgery. The skin lesions of these two conditions are distinct and do not usually occur simultaneously. SN is associated with a poor prognosis. ( info) |
| We cared for four neonates with transient hyperammonemia of the newborn (THAN), all of whom exhibited sclerema during their course. Sclerema is a hardening of the subcutaneous tissues, which gives the tissue a firm, rubber-like quality. This association has not been previously reported, and it may provide a clue to early diagnosis and intervention in THAN. ( info) |
4/10. sclerema neonatorum and joint contractures at birth as a potential complication of chronic in utero hypoxia. Three term neonates presented with an unusual combination of skin changes (sclerema) and joint contractures at birth. In each case a prolonged period of fetal hyporeactivity was reported by the mother. Each child demonstrated a number of clinical findings consistent with chronic fetal hypoxia. Each child recovered with normal joint function. We postulate that sclerema neonatorum and joint contractures are another, though poorly recognized sequela of chronic in utero hypoxia and are preceded by a prolonged period of diminished fetal activity. ( info) |
5/10. 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. ( info) |
| A female newborn infant whose illness was diagnosed as sclerema edematosum on the basis of diffuse edematous skin induration having rapidly occurred until her death on the third day of life, was pathologically investigated. autopsy revealed notable interstitial fibrosis of the subcuteneous adipose tissue, variable increases of fibrous connective tissue in various visceral organs, particularly prominent in the alimentary tract, as well as endocardial fibroelastosis of the heart. Although the skin lesion is similar to that of sclerema neonatorum presented by HUGHES et al., systemic fibrosis in the visceral organs has not been reported in the literature with respect of this disease. Furthermore, this case presented striking histopathological differences from scleroderma neonatorum and infantile subcutaneous fat necrosis in many respects. It is conceivable that this case is a peculiar disorder at least pathologically different from the previously known cutaneous indurative disorders of newborn infants. Possible pathogenesis of this case was briefly discussed. ( info) |
| The clinical presentation and diagnostic evaluation of a septic neonate with sclerema neonatorum and impending tissue loss secondary to compartment syndromes is presented. Vascular disease leading to tissue loss is uncommon in children. To our knowledge, this is the only reported case of a compartment syndrome leading to tissue loss in a neonate. ( info) |
| subcutaneous fat necrosis of the newborn (SFNN) developed in a 1-week-old black boy. His mother had received numerous medications for eclampsia. Birth was by Caesarean section and complicated by meconium aspiration. There were numerous nodules over the back, buttocks and extremities that yielded a caseous-like material. Microscopically, these nodules showed crystallization and necrosis of the fat. hypoglycemia, pneumonia, oliguria, thrombocytopenia, seizures and urinary infection were associated with the cutaneous problem and led to a fatal outcome 2 weeks after birth. ( info) |
9/10. Unusual presentation of cytomegaloviral infection in a 5-month-old baby: case report. Painful restricted movements of the extremities, hyperpigmentation over swollen joints, and a sclerema-like feel to the skin with increased serum triglyceride was seen in a 5-month-old baby with postnatal CMV infection. In an infant with pseudoparalysis of the limbs, the possibility of CMV infection has to be considered. ( info) |
10/10. A case of Hutchinson-Gilford progeria syndrome mimicking scleredema in early infancy. We report a case of Hutchinson-Gilford progeria syndrome (HGPS). The patient showed the characteristics of scleredema at the age of 2.5 months but developed all the manifestations of HGPS gradually until 10 months old. The possibility of development of HGPS should by considered in any case of scleredema at birth or in early infancy. ( info) |