Buschke–Ollendorff syndrome
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Buschke–Ollendorff syndrome (BOS) is a rare genetic skin disorder associated with LEMD3, that typically presents with widespread painless papules.[1][5]
Quick Facts Other names, Symptoms ...
Buschke–Ollendorff sign | |
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Other names | Dermatofibrosis lenticularis disseminata[1] |
Buschke–Ollendorff syndrome has an autosomal dominant pattern of inheritance. | |
Symptoms | Papules in skin[1] |
Causes | Mutations in the LEMD3 gene.[2] |
Diagnostic method | X-ray, ultrasound[3] |
Treatment | Surgery for hearing loss(or complications)[4] |
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It is inherited in an autosomal dominant manner.[6][7] Conditions that may appear similar include tuberous sclerosis, pseudoxanthoma elasticum, neurofibroma, and lipoma, among others.[1]
Its frequency is almost 1 case per every 20,000 people, and it is equally found in both males and females.[4] It is named for Abraham Buschke and Helene Ollendorff Curth, who described the condition in one female in 1928.[1][8]