Osteopoikilosis Associated With Dermatofibrosis Lenticularis Disseminata

Authors:
  • LDLis Danielsen
    Lis Danielsen
  • KMKnud Midtgaard
    Knud Midtgaard
  • HCHans Ewald Christensen
    Hans Ewald Christensen

Abstract
We report a patient with osteopoikilosis and dermatofibrosis lenticularis disseminata with unusual flexural location. The patient was undersized, had diabetes mellitus, and pronounced arteriosclerotic changes. Histologic examination showed the cutaneous lesions sharply demarcated with a pseudotumorous appearance. Electron microscopic studies of these lesions revealed some of the elastic fibers broken up, partly into big lumps consisting of the amorphous component, partly into smaller granular elements, presumably representing the fibrillar component. The collagen fibers were normal. A certain resemblance to pseudoxanthoma elasticum is discussed.