Dermatofibrosis Lenticularis

Description: Background: Buschke-Ollendorff syndrome is a rare hereditary disorder of connective tissue. It is inherited as a pleiotropic autosomal dominant trait with incomplete penetrance. Albers-Schönberg described this syndrome in 1915, and Buschke and Mme Ollendorff described it in 1928. Buschke-Ollendorff is considered to be a hamartoma, an association of osteopoikilosis and connective tissue nevi. Pathophysiology: In the presence of 10% calf serum, cultured fibroblasts of patients with Buschke-Ollendorff syndrome produce 3-8 times more tropoelastin than fibroblasts of healthy individuals. Elastin production is higher in involved and uninvolved skin. Elevated elastin messenger ribonucleic acid (mRNA) levels suggest that Buschke-Ollendorff syndrome may result from abnormal regulation of extracellular matrix, leading to increased levels of elastin mRNA and increased accumulation of elastin in the dermis. Frequency: Internationally: The incidence (1 case per 20,000 population) is rare. The prevalence of Buschke-Ollendorff syndrome is rare throughout the world. Mortality/Morbidity: The cause of mortality may be malignancy of bone densities into osteosarcoma, chondrosarcoma, and giant cell tumor. The otosclerosis with hearing impairment, stenosis of the aortae, and diabetes frequently found in this syndrome make the patient's condition serious. Department of Dermatology, Venereology and Allergology, University of Medicine at Wroclaw, Poland