Sclerema neonatorum associated with systemic fibrosis and endocardial fibroelastosis

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.