Buschke Ollendorff syndrome

Overview

A rare skin disease characterised by a firm, flesh-coloured or slightly yellowish eruption distributed symmetrically on the trunk and extremities, and induration of the skin and subcutaneous tissues. Foci of dense bone seen on x-rays, particularly in the pelvis and extremities, are the principal symptoms of osteopoikilosis. It is a sequelae to some type of acute infectious process. Both sexes affected. Inheritance is autosomal dominant with high penetrance and variability of expression.

Causes

The syndrome is inherited as an autosomal dominant variant with incomplete penetrance.