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. They usually are located on the trunk and proximal extremities, and skin folds often are involved. The lesions are painless, nonpruritic, and enlarge with the growth of the child. The dermal lesions are composed of collagen and elastin fibers, and in some instances, mucopolysaccharides.