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Glomuvenous malformation | DermNet New Zealand

Glomuvenous malformation

Author: Dr Marius Rademaker, Dermatologist, Hamilton, New Zealand, 2008. Update by Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand. December 2017.


What is a glomovenous malformation?

Glomuvenous malformation is an unusual form of venous malformation, typically diagnosed at birth or later in infancy or childhood. Glomuvenous malformation is also known as glomangioma or glomangiomatosis.

Who gets glomovenous malformation? 

Although glomovenous malformation may be sporadic, glomovenous malformation may be familial with an autosomal dominant pattern of inheritance (this is where half the children of an affected parent inherit the condition). An abnormal chromosome 1p21–p22 has been identified in these families. It results in inactivation of a protein called glomulin.

What are the clinical features of glomovenous malformation?

Glomuvenous malformation presents as a localised or segmental collection of soft, pink, red or blue nodules or coalescing plaques. They may arise anywhere on the skin and may rarely affect mucous membranes. These vascular lesions are less compressible than other forms of venous malformation

Lesions tend to get more widespread, thicker, and deeper in colour with age. Although the nodules start off painless, they may later become tender to touch.  

How is glomovenous malformation diagnosed?

Glomovenous malformation may be suspected by its clinical appearance, but may undergo biopsyHistology resembles venous malformation with large, dilated, thin-walled veins in the dermis and subcutaneous tissue. Clusters of α-actin-positive glomus cells are found lining the venous spaces. 

How is glomovenous malformation treated?

Surgical excision of glomuvenous malformation is difficult. Treatment options include laser and sclerotherapy.

 

Related Information

References

  • Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, Burrows P, Frieden IJ, Garzon MC, Lopez-Gutierrez JC, Lord DJ, Mitchel S, Powell J, Prendiville J, Vikkula M; ISSVA Board and Scientific Committee. Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies. Pediatrics. 2015 Jul;136(1):e203-14. doi:10.1542/peds.2014-3673. Epub 2015 Jun 8. Review. PubMed PMID: 26055853. Journal.

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