Buschke–Ollendorff syndrome

Author: Brian Wu, MD candidate, Keck School of Medicine, Los Angeles, USA. DermNet New Zealand Editor in Chief: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. December 2016.

What is Buschke–Ollendorff syndrome?

Buschke–Ollendorff syndrome refers to a rare, hereditary disorder affected the connective tissues. It is also known as dermatofibrosis lenticularis disseminata, dermato-osteopoikilosis and familial cutaneous collagenoma.

Who gets Buschke–Ollendorff syndrome?

Buschke–Ollendorff syndrome occurs in 1 in every 20,000 live births worldwide. There appears to be no racial, sexual or geographical predilection. Lesions are present at birth.

What causes Buschke–Ollendorff syndrome?

Buschke–Ollendorff syndrome is a genetic disease. The underlying cause of Buschke–Ollendorff syndrome is mutation of the LEMD3 gene, which provides instructions for the making of proteins that support connective tissues.

What are the clinical features of Buschke–Ollendorff syndrome?

Buschke–Ollendorff syndrome is characterised by small connective tissue naevi.

Non-cutaneous but significant features of Buschke–Ollendorff syndrome include:

How is Buschke–Ollendorff syndrome treated?

The treatment of Buschke–Ollendorff syndrome may involve:

What is the outcome for Buschke–Ollendorff syndrome?

The outcome for Buschke–Ollendorff syndrome depends on the associated disorders.

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