Juvenile elastoma and osteopoikilosis (the Buschke-Ollendorff syndrome)

Abstract

Sixteen patients from 7 different families with the Buschke-Ollendorff syndrome were studied. Osteopoikilosis was found in 2/3 of the patients radiologically examined and all but 2 had skin involvement. The predominant clinical pattern consisted of grouped skin colored papules and discs that were distributed asymmetrically and which usually were presented at an early age. The skin lesions showed the characteristic histological changes of juvenile elastoma which, it was suggested, was the specific dermatological abnormality of the Buschke-Ollendorff syndrome.