Hereditary Osteolysis

Abstract

Three children and their father were followed from the onset of arthritic symptoms in early childhood through years of progressive lysis of the carpal, tarsal and adjacent bones. The family exhibited a peculiar Marfan-like appearance with frontal bossing and micrognathia, mild scoliosis, pes cavus, plantar cysts, and frequently, changes in the elbows. A variable hyperinflam-matory response to trauma and stress was noted. Chemical and histologic indices of tissue destruction were evaluated.