[Adiponecrosis subcutanea neonatorum--sclerema neonatorum--Buschke's scleredema. Nosography with reference to the literature since 1950].
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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.
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.
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.
Specimens from four cases of sclerema neonatorum were studied by light and electron microscopy. The connective tissue bands of the cutis and subcutis were thickened and alternated with loosely distributed bands in broad, distended areas of basal substance. Extensive areas areas filled with cross-banded structures were observed in the dermis. In the interlobular septa of the subcutaneous and perirenal fat splitting of fibers into microfibrils was visible. Changes in the capillaries and small blood vessels were also noted. Neither fat cells filled with rosettes of needle-like crystals, nor any inflammatory infiltrates of foreign body type giant cells were found in subcutaneous tissue.
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.
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High-risk infants are particularly prone to sepsis which can be accompanied by sclerema neonatorum. This condition is manifested by skin hardening. Causation of hardening of a sick infant's skin must be identified for appropriate treatment to be implemented. Nursing care of the infant with sclerema is very complex. Throughout the course of the disease, the infant frequently requires antimicrobial therapy, ventilatory support, exchange transfusions, precise intake and output measurements, and temperature control. Psychosocial support for the family and infant is also an important part of the nurse's role. In spite of standard therapy, the mortality rate for infants with sclerema remains high. New advances, such as exchange transfusions, give some hope for the survival of these very ill infants.
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.
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