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