Clinical and morphologic findings in 3 sibs with congenital cutis laxa are presented. A severe urinary malformation in one affected infant is reported in detail. Elevated serum copper concentrations were observed in 2 of the sibs and in the healthy mother. However, the 64Cu uptake of fibroblast cells from tissue culture was not increased. Ultrastructural pathologic findings from skin biopsies have been studied and compared at birth and at age 2 years. The lack of junction between the 2 elastic fiber components was similar. Further evidence for clinical heterogeneity of this disease is stressed.
In their study of a case of lethal midline-granuloma, the authors describe the relations between this affection, Wegener’s granulomatosis and lymphoma. The case under review illustrates the difficulty in accurately defining the nosologic picture of the disease. Anatomo-pathological examinations present two possibilities: one being malignant granuloma of the Stewart type, the other that of lymphoma. From its subsequent evolution, we may eventually learn whether this is purely a local illness or represents the onset of a systemic disease.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.