[Osteopoikilia with dermofibrosis lenticularis disseminata (Buschke-Ollendorf syndrome) (author's transl)].
In a family, we have observed through 3 generations a Buschke-Ollendorf syndrome. All 12 children and grandchildren, descendants from actually 59 year old grandmother were examined. 8 probands had a osteopoikilia, 5 of them as the grandmother had a dermatofibrosis lentcularis disseminata. The Buschke-Ollendorf syndrome is not really a syndrome in his strong sense, rather a variant of osteopoikilia with participation of skin.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005350:Fibroma / Q000235:genetics*; D005528:Foot / Q000000981:diagnostic imaging; D006225:Hand / Q000000981:diagnostic imaging; D006330:Heart Defects, Congenital / Q000150:complications; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010023:Osteopoikilosis / Q000000981:diagnostic imaging / Q000235:genetics; D010026:Osteosclerosis / Q000235:genetics*; D010375:Pedigree; D011859:Radiography; D012857:Situs Inversus; D012878:Skin Neoplasms / Q000235:genetics*; D013154:Spleen / Q000002:abnormalities
Osteopoikilie mit Dermatofibrosis lenticularis disseminata (Buschke-Ollendorf-Syndrom)