Elastin production is higher in involved and uninvolved skin. This leads to increased levels of elastin mRNA and increased accumulation of elastin in the dermis. Prevalence of Buschke-Ollendorff syndrome is rare throughout the world. Otosclerosis with hearing impairment, stenosis of aortae, and diabetes frequently found in this syndrome make the patient s condition serious. No racial predilection exists for Buschke-Ollendorff.
Incidence is equal for males and females.
Other factors of the syndrome are revealed with time. The coexistence of cutaneous lesions, such as disseminated lenticular dermatofibrosis, and skeletal changes, such as osteopoikilosis, characterize Buschke-Ollendorff syndrome. Cutaneous lesions appear in childhood, often in the first year of life. They may be observed in the fetus. Rarely, they may occur in adulthood.