Abstract

Buschke–Ollendorff syndrome, also known as dermatofibrosis lenticularis disseminata, is a rare genetic disorder associated with LEMD3. It is believed to be inherited in an autosomal dominant manner.It is named for Abraham Buschke and Helene Ollendorff Curth who described it in a 45-year-old woman. Its frequency is almost 1 case per every 20,000 people and is equally found in both males and females.

Symptoms and signs

The signs and symptoms of this condition are consistent with the following{it should be noted that possible complications include aortic stenosis and hearing loss):

Diagnosis

The diagnosis of this condition can be ascertained via several techniques one such method is genetic testing, as well as:

- X-ray

- Ultrasound

- Histological test

Diagnosis | Differential diagnosis

The DDx for an individual believed to have Buschke–Ollendorff syndrome is the following:

- Melorheostosis

- Sclerotic bone metastases.

Treatment

In terms of the treatment of Buschke–Ollendorff syndrome, should the complication of aortic stenosis occur then surgery may be required.