Bilateral macrodystrophia lipomatosa of the upper extremities with syndactyly and multiple lipomas

Abstract

Macrodystrophia lipomatosa is a rare disease that causes congenital local gigantism of part of an extremity, which is characterised by an increase in all mesenchymal elements, particularly fibroadipose tissue. This is the first report to our knowledge of a case of histologically confirmed bilateral macrodystrophia lipomatosa of the upper extremities with syndactyly and multiple lipomas.

Key Words::

• Bilateral macrodystrophia lipomatosa
• gigantism
• hand tumours
• lipomas
• syndactyly

Acknowledgements

We thank Dr. Rinze Wolf, radiologist of the Medical Center Leeuwarden, The Netherlands for his help with making and analysing the computed tomograms and his valuable discussions.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.