[Dermatofibrosis lenticularis disseminata with osteopoikilosis (Buschke-Ollendorff syndrome)]

Presented are 4 cases showing combined occurrence of lenticular disseminated dermatofibrosis and osteopoikilosis (Buschke-Ollendorff syndrome). Histological findings of the skin show localized increases in elastic and collageneous structures. Type I of the Buschke-Ollendorff syndrome which is characterized by disseminated small pepper-corn like changes in the skin must be differentiated from type II which is named "dermatofibrosis nodularis xanthomatoides multilokularis" with osteopoikilosis, showing larger, single or plaque like connected changes of the skin. The Buschke-Ollendorff syndrome is a congenital autosomal dominant hereditary abnormality arising from the mutual mesodermal genesis of skin and bone changes. The Buschke-Ollendorff syndrome occasionally brings about impaired growth and mental retardation as well as rheumatoid complaints. Osteopoikilosis always appears symmetrically showing different changes in the bones usually without changes in the skin; in the contrary the lenticular disseminated dermatofibrosis is always occurring combined with findings of osteopoikilosis. During the growing years we observed patients with an increase in size and density of the bone changes and also new lesions, while the skin changes remained nearly the same.